2016 PULMON Setting SEP - DEC ISSUE - Apccm … · Acute progressive type usually leads to higher mortality than chronic subtype. The frequency and pattern of pulmonary involvement

Pulmon, Vol. 18, Issue 3, Sep - Dec 2016 91

Editorial

95 Pulmonary manifestations in Systemic Autoimmune DiseasesWhat a pulmonologist must know?Vishnu Sharma M.

Review Article

100 Paediatric SpirometeryPaulo Varghese Akkara

Original Article

108 Pattern of Interstitial Pneumonitis in Patients with Systemic SclerosisHarsha P.V.

Radiology Quiz

114 Vishnu Sharma M.

Case Reports

116 An Unusual Cause of Chest PainArjun P.

118 A Case Report of Young’s Syndrome and Review of LiteratureVenu Gopal Panicker

121 Lung Abscess Mimic- A Case ReportRavindran Chetambath

124 Renal Transplant Recipient with CoughAmrutha Mukundan

Guidelines for authors

92 Pulmon, Vol. 18, Issue 3, Sep - Dec 2016




Editorial

Pulmonary manifestations in Systemic Autoimmune Diseases -What a pulmonologist must know?

Vishnu Sharma M.Professor and head, Dept. of Respiratory MedicineA J Institute of Medical Sciences,Mangalore, Karnataka - 575 004Phone : 0824 2216321Mobile : +91 9448126321E-mail : [email protected]

Autoimmune diseases are a group of disorders where immunesystem malfunctions and attacks one’s own tissues in the body.Autoimmune diseases are characterized by antibodies that aredirected against self-antigens (auto antibodies). Autoimmunediseases can be divided into two general groups, Organ specific, wherethe auto antibodies attack a specific organ and Non-organ specific (orsystemic), where the auto antibodies attack multiple organ systems1.Systemic autoimmune diseases include Rheumatoid Arthritis (RA),Systemic Lupus Erythematosus (SLE) (and subsets of Lupus), Sjögren’ssyndrome (SjS), Systemic Sclerosis (SSc), Polymyositis (PM) andDermatomyositis (DM), Mixed connective tissue disease,Granulomatosis with Polyangiitis (Wegener's granulomatosis), Churg-Strauss syndrome, Good pasture’s syndrome and Ankylosingspondylitis1.

Involvement of vital organs, mainly lungs and kidneys in systemicautoimmune diseases is common. Usually this leads to progressivedeterioration in the function of the involved organ. One of the mostcommon causes of death in systemic autoimmune diseases is due toinvolvement of lungs and kidneys2.

Involvement of lung is a major contributor to morbidity andmortality in systemic auto immune diseases. The incidence ofpulmonary involvement varies from 18 to 61%. Pulmonarymanifestations in systemic auto immune disease can be due to chronic


immune activation leading to tissue damage, increased susceptibilityto infections (often related to immunosuppressive medications ordue to reduced immunity as a part of the auto immune diseaseprocess) or direct toxicity from immunosuppressive therapy or acombination of all these factors3. Pulmonologists should be able toidentify and diagnose the cause for pulmonary manifestations ofsystemic auto immune disease.

Pulmonary complications generally occur in patients with wellestablished auto immune diseases, usually after a few years ofsystemic manifestation. Rarely some patients may initially presentwith pulmonary symptoms without any systemic manifestations3.Hence pulmonologists should be aware about the diverse pulmonarymanifestations of systemic auto immune diseases and should be ableto evaluate for systemic autoimmune disease in a patient withsuspected pulmonary manifestation due to systemic autoimmunedisease. Systemic auto immune diseases should be considered asone of the differential diagnosis in a patient with pulmonary diseaseof unknown etiology3.

Systemic autoimmune diseases can lead to pulmonaryparenchymal disease (interstitial lung disease (ILD)), inflammationof the pleura (pleural thickening and effusions), airways and pulmonaryvasculature (vasculitis and pulmonary hypertension), involvementof respiratory muscles and thoracic cage4. All patients with systemicauto immune diseases should be enquired regarding any respiratorysymptoms and any respiratory symptom should be evaluatedproperly to determine the cause and severity of involvement.

Pulmonary function tests including the diffusing capacity forcarbon monoxide (DLCO) and high resolution CT scan of chest (HRCT)are the investigations to detect pulmonary involvement3. Prognosisvaries depending on the site, type and severity of involvement.Pulmonary involvement should be detected early and properimmunosuppressive therapy should be instituted. Appropriatetreatment improves the lung function and improves the long termsurvival in these patients3.

The frequency and pattern of pulmonary involvement,management and prognosis vary in different systemic autoimmunediseases. Most common pulmonary manifestation in systemic autoimmune disease is ILD3. This should be differentiated from respiratoryinfection and medication induced lung injury. Recent studies suggestthat the incidence of ILD is increasing in patients with systemic autoimmune disease. This may be due to increased use of diagnosticmodalities and increased survival of these patients due to bettertreatment. The reported rate of radiographic prevalence of subclinicalILD in systemic auto immune disease ranges from 33% to 57%4.

Vishnu Sharma M. - Pulmonary manifestations in Systemic Autoimmune Diseases -What a pulmonologist must know?


Systemic auto immune related ILD have a better prognosis thanidiopathic ILD5. Usually, they have NSIP pattern, respond to immunosuppressive therapy and progression is slower than IPF. Butrheumatoid arthritis related ILD with UIP pattern has poor prognosis5.The median survival of all patients with rheumatoid arthritis relatedILD has been reported to be approximately 5 years. Mortality is highin patients who develop ILD with concomitant pulmonary hypertensiondue to pulmonary vascular involvement. In patients with Rheumatoidarthritis and SLE who develop ILD, mortality is 3-4 times higher thanthose without pulmonary involvement. Patients with polymyositis/dermatomyositis and systemic sclerosis with pulmonary involvementalso have higher mortality than other auto immune diseases6. Acuteprogressive type usually leads to higher mortality than chronicsubtype.

The frequency and pattern of pulmonary involvement vary indifferent systemic autoimmune diseases. The most commonpulmonary manifestation in SLE is unilateral or bilateral pleuraleffusion which is frequently associated with pericardial effusion.Pulmonary infections are also common in SLE7.

Rheumatoid arthritis is the most prevalent systemic auto immunedisease. Lung disease is a leading cause of death in Rheumatoidarthritis, second only to infection. ILD is the most common pulmonarymanifestation of rheumatoid arthritis8. Most common pattern is usualinterstitial pneumonia (UIP), which occurs in 40–62% of cases. Theprognosis for patients with Rheumatoid arthritis who have UIP is poor.This is a notable difference from other connective tissue disorders, inwhich a nonspecific interstitial pneumonia (NSIP) pattern is mostfrequently seen9. NSIP is the second most common pattern in patientswith rheumatoid arthritis, occurring in ~11–32% of patients. Pleuraleffusion is also common in rheumatoid arthritis. Rheumatoid pleuraleffusion is usually unilateral, more common in older males and inpatients with rheumatoid nodules. Pleural fluid is usually sterile,exudative with low pH (<7.3), low glucose (<60 mg/dl) and elevatedlactate dehydrogenase10.

Most common pulmonary manifestation in Systemic sclerosis,Sjögren's syndrome and mixed connective tissue disease is interstialfibrosis1. Amongst the systemic auto immune diseases systemicsclerosis is most often associated with PAH11. The most commonmanifestation in Polymyositis/dermatomyositis is aspirationpneumonia secondary to pharyngeal muscle weakness. In Sjögren'ssyndrome there is an increased prevalence of lymphocytic interstitialpneumonitis involving the lower lobes. The most commonmanifestation in Granulomatosis with Polyangiitis (Wegener'sgranulomatosis) is multiple nodules or masses with irregular marginsthat frequently cavitate and may be mistaken for pulmonarytuberculosis and other infectious diseases. Alveolar hemorrhage may



also be seen in Granulomatosis with Polyangiitis1. Pulmonary infiltrates,which may wax and wane, are often misdiagnosed initially aspneumonia in Granulomatosis with Polyangiitis.

In Churg-Strauss syndrome lung is the most common organinvolved1. More than 90% of patients with Churg-Strauss syndrome(CSS) have asthma. They typically present with asthma, peripheraleosinophelia, fever, and allergic rhinitis.

In Good pasture’s syndrome lung and renal involvement occurssimultaneously1. Good pasture's syndrome is characterized by a triadof diffuse pulmonary hemorrhage, glomerulonephritis, and circulatingantiglomerular basement membrane antibodies. Pulmonarypresentation usually consists of cough, mild shortness of breath,hemoptysis, and hypoxia in young male patients. Good pasture'ssyndrome is associated with extensive bilateral air-space consolidationand can be mistaken for pneumonia with multi organ dysfunction.

Among systemic auto immune diseases Ankylosing spondylitishad the mildest form of pulmonary involvement1. It leads to apicalfibrosis, ILD, emphysema, bronchiectasis and pleural thickening.Usually these changes are very mild.

Treatment with anti-inflammatory and/or immunosuppressiveagents is recommended regardless of the pattern of pulmonaryinvolvement3. Corticosteroids are the mainstay of therapy.Cyclophosphamide and azathioprine have been used with varyingsuccess.

Table 1 : Pulmonary manifestations in systemic auto immune diseases3.


The number of + signs indicates relative prevalence of each manifestation.DAH = diffuse alveolar hemorrhage.MCTD Mixed connective tissue disease

Systemic sclerosis + + + - - + + + -

Dermatomyositis/Polymyositis + + + - - + -

Rheumatoid arthritis + + + + + + + -

Sjögren’s syndrome + + + + + + + -

Systemic lupus erythematosus + + + + + + + + +

MCTD + + + + + + -

ILD Airways Pleural Vascular DAH



References

1. M. COJOCARU, Inimioara Mihaela COJOCARU, Isabela SILOSI,Camelia Doina VRABIE, Pulmonary Manifestations of SystemicAutoimmune Diseases; Maedica A Journal of Clinical Medicine,2011Volume 6; No.3, 224 -229

2. T. Mimori, R. Nakashima, and Y. Hosono, “Interstitial lung diseasein myositis: clinical subsets, biomarkers, and treatment,” CurrentRheumatology Reports, 2012, vol. 14, no. 3, 264–274

3. Te-Wei Hseun, and Ren-Guang Wu; Pulmonary Manifestations ofConnective Tissue Diseases; Journal of Internal Medicine ofTaiwan, 2015:26:177-185

4. Frankel SK, Brown KK. Collagen Vascular Diseases of the Lung.Clin Pulm Med 2006; 13: 25-36.

5. Castelino FV, Varga J. Interstitial lung disease in connective tissuediseases: evolving concepts of pathogenesis and management.Arthritis Res Ther. 2010; 12(4): 213.

6. Chen IJ, Jan Wu YJ, Lin CW, et al. Interstitial lung disease inpolymyositis and dermatomyositis. Clin Rheumatol 2009; 28: 639-646.

7. Pego-Reigosa JM, Medeiros DA, Osenberg DA. Respiratorymanifestations of systemic lupus erythematosus: old and newconcepts. Best Pract Res Clin heumatol. 2009;23: 460–480.

8. Doyle TJ, Lee JS, Dellaripa PF, et al. A roadmap to promote clinicaland translational research in rheumatoid arthritis-associatedinterstitial lung disease. Chest 2014; 145: 454–463

9. de Lauretis A, Veeraraghavan S, Renzoni E. Review series: aspectsof interstitial lung disease: connective tissue disease-associatedinterstitial lung disease: how does it differ from IPF? How shouldthe clinical approach differ? Chron Respir Dis 2011; 8: 53–82.

10. Avnon LS, Abu-Shakra M, Flusser D, et al. Pleural effusionassociated with rheumatoid arthritis: what cell predominance toanticipate? Rheumatol Int 2007; 27: 919–925.

11. Varda J Abraham D. – Systemic sclerosis: a prototypic multisystemfibrotic disorder. J Clin Invest 2007; 117:557-67.

12. Bouros D, Pneumatikos I, Tzouvelekis A. Pleural involvement insystemic autoimmune disorders. Respiration. 2008;75: 361–71.


Review Article

Paediatric Spirometery

Paulo Varghese AkkaraAssistant ProfessorDepartment of Pulmonary MedicineGovt. Medical College, Kozhikode

Introduction

Pulmonary diseases form a major cause ofchildhood morbidity and mortality and also a commonreason for paediatric outpatient visits and hospitaladmissions. Pulmonary function assessment thusbecomes a necessary investigation in the evaluationpathway of paediatric respiratory diseases. It measuresthe rate of changing lung volumes during forcedbreathing manoeuvres. In simpler terms it measuresthe airflow during inspiration and expiration. It is thepreferred method for assessing pulmonary function dueto its accuracy and the ability to diagnose acute andchronic lung disease in children.

Children have a dynamic developmental phaseduring which lung volume and airway size change withincreasing age. Spirometric parameters in children areinfluenced by height, age, sex, environmental factors,weight, ethnicity, prematurity, patient cooperation, effortand technical factors1, 2. Even though spirometry is themost widely performed pulmonary function test inadults, it is underutilized in the paediatric age group. Inpaediatric primary care physicians who were dealingasthmatic children particularly, it was found that onlyhalf were using spirometry and out of that only halfwere properly interpreting the spirometry results.3

Spirometry was considered to be a difficultprocedure to perform in preschool age group when

compared to children above 6 yrs. With access to betterspirometry equipment and modified criteria foracceptability and repeatability, it is possible to performreliable spirometry tests even in preschool children bytrained personnel.4

Indications of Spirometry

In paediatric age group, it is indicated for theevaluation of symptoms and signs of respiratoryobstruction verses respiratory restrictive diseases(Table 1), measure the severity of the disease, determinethe effect of medication on the disease, assess lungfunction preoperatively, monitor the disease over time,and rule out other causes of wheezing.6,7,11

Table 1 :Obstructive and restrictive lung diseases

Obstructive Lung Disease Restrictive Lung Disease

Asthma8 Interstitial lung disease

Bronchiectasis Obesity

Cystic fibrosis9 Neuromuscular disorders

Subglottic/Tracheal stenosis Sarcoidosis

Tracheomalacia Scoliosis

Vocal cord dysfunction PectusExcavatum

Keywords : Spirometery, Spirometry in children, Paediatric Spirometery


Paulo Varghese Akkara - Paediatric Spirometery

The goals of spirometry for children withasthma are to provide an objective measurement oflung function, assess the degree of obstruction andreversibility to bronchodilator, evaluate the responseto therapy, and assist with the decision to step up orstep down therapy

Spirometry is helpful in ascertainingpreoperative lung function in flaccid neuro muscularscoliosis like cerebral palsy.10 Field spirometry canbe used to screen schoolchildren for respiratorydiseases.7

Contraindications to spirometry

Contraindications for performing spirometryare almost same as in adult population. It include recentthoracic, abdominal or eye surgery, persistent cough,altered mental capacity or inability to follow instructions,recent pneumothorax or myocardial infarction (withinone month), or inability to adequately seal themouthpiece, hemoptysis of unknown origin, aneurysm,uncontrolled hypertension, nausea, vomiting andconfusion or dementia.5,6,12 Suboptimal testing mayoccur if the individual being tested has chest orabdominal pain, oral pain, urinary incontinence, hasbeen smoking or consumed alcohol within hours of thetest, or consumed a large meal within two hours of thetest.6,12

Spirometry Testing

In the past, spirometry has been performed inpulmonary clinics, but more recently, spirometry hasbeen done in primary care practices to diagnose andmonitor children with asthma. Basic handheldspirometers provide forced expiratory volume in onesecond (FEV1) and forced vital capacity (FVC) valuesthat can be compared manually with available predictednormal values. The newer electronic spirometerscalculate the percentage of the predicted normal valuesbased on reference values already programmed afterentering patient details and performing the test. Theequipment should fulfil American Thoracic Society/European Respiratory Society (ATS/ERS)recommendations for spirometry.5, 6

The calibration, maintenance, and infectioncontrol measures should be performed as per

recommendations of the manufacturer of theequipment. Personnel performing spirometry needto be trained in the use and calibration of theequipment to ensure accuracy. The health careprovider offering interpretations should also beproperly trained in identifying common errors andto interpret the results of spirometry. The respiratorytechnician or physician performing the spirometrytest has to do at least five tests a week for maintainingcompetency for test performance.5 The variousterminologies related to spirometry result aredescribed in Table 2.

Table 1

Terminology Description

FVC Total amount of air exhaledout ‘fast’ following a maxi-mal inhalation

FEV1 Amount of air exhaled outduring the 1st second of aforced exhalation followingmaximal inhalation

FEF25-75, MEF50 Forced expiratory flow overmiddle half of the FVC –that is, average flow from25% of the FVCto 75% ofthe FVC during forced ex-piration. It is a sensitive in-dex of airflow obstruction

FEV1/FVC ratio Ratio of FEV1 and FVC.

PEFR Measurement of fastestexpiratory flow measuredby a spirometer or peakflow meter.It is measuredin L/min by portable peakflow meters and in L/s byspirometers

FET Total time taken for a pa-tient to complete their ex-halation in a FVC manoeu-vre

(forced expiratoryvolume in one second)

(forced vital capacity)

(peak expiratory flowrate) or PEF

(forced expiratory time)

(maximum mid-expira-tory flow)



How to perform the test

The spirometry test technique is similar inadults and children aged >6 years. The procedure shouldbe explained to the child in a friendly way. Patients shouldbe asked if they have recently taken any medicationssuch as bronchodilators, when they last had a meal andthey should be advised to avoid tight clothes that couldinterfere with the test. The patient’s weight and heightshould be measured and entered into software alongwith the name, ID, age, sex, and race. The position ofthe patient for the test may be sitting (preferred) orstanding.

Additional training is needed on how to coachchildren before and during the procedure with a dem-onstration of the steps required for successful testing.Children should practice using the mouthpiece and thenose clip so they will be cooperative and able to followthe directions for testing. Testing requires the child toexhale forcefully and then take a rapid deep breath withhis or her mouth forming a tight seal around the mouth-piece of the spirometer without breathing through theirnose. Children will often need to practice this techniquebefore actual testing can be accomplished.

FVC test manoeuvre

The child is asked for 2-3 tidal breaths in andout and to inhale deeply with the lips sealed tightlyaround the mouthpiece. The child is then asked to blowair through the mouthpiece as fast as possible and tocontinue to blow until no air is left to exhale. Duringinstructions to children, some phrases may be used suchas “sucking on a straw” for a deep inspiration, “blowingout birthday candles”for a forceful expiration, and “keepblowing and keep blowing” for complete exhalation un-til no air is left.12 The test should be repeated at leastthree times and checked for acceptability and repeat-ability criteria (Table 3). If the results are not accept-able, the test may be repeated a maximum of eighttimes before abandoning it. During the test it is essen-tial to ensure that the child is cooperative and followsthe instructions, that there is a tight seal around themouthpiece, the mouthpiece is not obstructed, inhala-tion is complete, the start is fast and forceful, there is nopause, no other breath or cough during exhalation, noexhalation through the nose, and the blow is continueduntil exhalation is complete, as suggested by a plateau

on the volume-time graph. Use of computer-generatedincentive graphics may be helpful to encourage pre-school children to perform spirometry.13 Although nosignificant differences in FEV1 or FVC measurementsin children with and without nose clips have been de-scribed, the use of a nose clip is recommended whileperforming spirometry to avoid part of the blow throughthe nose.14

Table 3 : Acceptability and repeatability criteria forspirometry maneuvers in children 6,12

Criteria for accepting individual spirograms(within-manoeuvre criteria):

1. They are free from artifactsGlottis closureCoughEarly termination of effortsPoor effortsLeakObstructed mouthpiece

2. They have good starts• For children of 6-12 years of age: extrapolatedvolume of <5% of FVC or <100mL if FVC<1000mL,whichever is greater.

3. They show satisfactory exhalation (end of testcriteria)• Duration of 3 seconds for children (>6 secondsfor adults) or a plateau in theVolume-time curveor if the subject cannot or should not continue toexhale. Plateau is defined as volume of less than25mL/s for at least 1s.

Criteria for repeatability (between-manoeuvre criteria):

1. After three acceptable spirograms have beenobtained, assess them for repeatability•

• The two largest values of FVC must be within150mL of each other or within 5% FVC or <100mLif FVC <1000mL

• The two largest values of FEV1 must be within5% FVC or <100mL if FVC <1000mL

• If both of these criteria are met, the test sessionmay be concluded

• If both of these criteria are not met, continuetesting until both of the criteria are met withanalysis of additional acceptable spirograms, ora total of eight tests have been performed, or thepatient is unable to continue.



How to interpret spirometry results

The steps for interpreting spirometry resultsinclude:(1) Assessing the tests for acceptability and repeat-

ability(2) Identifying the spirometry pattern (normal, ob-

structive, restrictive, or mixed)(3) Grading the severity of the pattern identified(4) Diagnosing and treating the condition or investi-

gating further

Assessment of acceptability and repeat-ability of tests

There are usually two types of graphs in spirom-etry, flow-volume loop (both expiratory and inspiratoryflow is recorded) and volume-time curve (also knownas spirogram) and both are useful for optimum qualitycontrol. If expiratory flow is only measured and plottedagainst volume it is called flow volume curve. The flow-volume loop is particularly useful for evaluating the ini-tial part of the FVC manoeuvre and the volume timecurve is useful for assessing the later part of the FVCmanoeuvre. (Figure 1A and 1B) Before assessing spiro-gram, any errors or artifacts in the spirometry proce-dure should be detected by looking at the shape of theflow-volume loop. The errors may include hesitation intest, premature finish (defined as flow ceasing at morethan 10% of peak flow), poor effort, and cough (Figure2). Individual spirograms are acceptable if they are freefrom errors or artifacts, have good starts, and satisfac-tory expiration in accordance with ATS/ERS standards.(Table 3)14.

Fig 1 A : A Flow volume loop

Fig 1 B : B Volume time curve

Fig 2 : Common errors in flow volume loop

In preschool children, some modifications inthe criteria have been suggested in an ATS/ERS state-ment on pulmonary function testing. In these children,an extrapolated volume of <80mL or 12.5% of the FVC isacceptable as a good start. The plateau is not defined inpreschool children as the criteria for satisfactory expi-ration (end of test) and the flow-volume curve demon-strating a rapid rise to peak flow and a smooth descend-ing limb is acceptable.15 For school age children a mini-mum of three satisfactory spirograms is required but,for preschool children, two acceptable spirograms aresufficient if the second highest FVC and FEV1 are within100mL or 10% of the highest value, whichever isgreater.6,15 Preschool children between the age of 3 and5 years require a short forced expiratory time as shortas 1.7 seconds.12,16 Therefore, the expert opinion is thatFEV0.5 and FEV0.75 may be used in preschool children.15


Identification of spirometry pattern

Abnormalities in a spirometry test can be iden-tified by looking at the shape of the curve and compar-ing the values of test parameters with reference valuessuitable for age, height, weight, sex, and ethnicity. Usu-ally the report will have two curves (a flow-volume loopand a volume-time curve) for both pre and post-bron-chodilator tests(if obstruction or airflow limitation ispresent) along with numerical values of spirometry pa-rameters with the percentage of predicted and percent-age change after the bronchodilator test. A samplespirometry with obstruction and bronchodilatorreversibility testing is given in Figure 3.

Fig 3 : Spirometry with obstruction andbronchodilator reversibility

Normal flow-volume curve looks like a sail whichascends sharply to a peak and then descends at an angleof about 45°. A concave curve indicates mild to moder-ate airway obstruction and an elongated finish like arat’s tail or dog leg pattern indicates severe obstruc-tion. A small flow-volume curve suggests a restrictivepattern and a flat FVC flow-volume curve without a peaksuggests intra thoracic obstruction.12 Younger childrenhave rapid emptying of the larger airways comparedwith a smaller lung volume that results in a convex shapeof the flow-volume curve, and the shape becomes morelinear as the child grows29. Various examples of flow vol-ume loop in different conditions are shown in Figure 4.

The commonly used parameters to interpretspirometry results are FEV1, FVC, and the FEV1/FVCratio. The FEV1/FVC ratio is the most sensitive and spe-cific indicator for identifying airway obstruction. Theseparameters are compared with reference values. Pae-diatric reference values have been published for differ-ent countries. 17, 18.

Spirometry results may suggest one of the fol-lowing four types of ventilation patterns: normal, ob-structive, restrictive, or mixed pattern.19, 20 Normal val-ues of FVC (>80% of predicted or above the lower limitof normal), FEV1 (>80% of predicted or above the lowerlimit of normal), and FEV1/FVC are suggestive of nor-mal spirometry.The obstructive pattern is usually char-acterized by decreased FEV1 (<80% of predicted or be-low the lower limit of normal), decreased FEV1/FVC,and normal FVC (FVC may be decreased in severe ob-struction).21 A value of mid expiratory flow (FEF25-75%)below 60% of predicted also suggests an obstructivepattern.22 FEF25-75% is thought to be less effort-depen-dent than FEV1 as it does not include high flows in thelung volume and is considered a measurement of smallairway patency.23 The restrictive pattern is suggestedby predominantly decreased FVC, normal or decreasedFEV1, and normal or increased FEV1/FVC. The propor-tionate reduction in both FEV1 and FVC is suggestive ofa restrictive pattern or poor patient effort. The mixedpattern has a decreased value of all three parameters(i.e. FEV1, FVC, and FEV1/FVC). Normal expiratory flowbut decreased inspiratory flow is suggestive of collaps-ible extra thoracic airway obstruction (e.g. laryngealparalysis) whereas decreased maximal expiratory flowwith normal inspiratory flow suggests collapsible majorintrathoracic airway obstruction. (Figure 4) If both in-spiratory and expiratory flows are decreased, a fixed


Fig 4 : Various pattern of flow volume loop



intrathoracic or extra thoracic airway obstructionislikely.24 Simple interpretation of spirometric data fromthe above mentioned parameters is shown as an algo-rithm in fig 5.

Fig 5 :Algorithm for spirometric interpretation

Bronchodilator Response

The bronchodilator response (BDR) may beused to assess reversibility of the obstructive pattern inspirometry. Before conducting a bronchodilator test it isnecessary to ensure that the child has not used a short-acting ß2-agonist for the preceding 4–6 hours, long-act-ing agonist for 12 hours and sustained release theo-phylline for 24 hours. The BDR is assessed after 10-15minutes of 200-400µgm of salbutamol inhalation using ametered dose inhaler with a spacer. The response tothe bronchodilator (FEV1 improvement>12% and 200mlin adults and >12% in children) suggests reversibility ofairway obstruction which is characteristic of asthma.25

A course of oral steroids for two weeks or inhaled ste-roids for three months can also be used to determinebronchodilator reversibility if asthma is strongly sus-pected. However, it is important to remember that anegative BDR on an individual test does not exclude thediagnosis of asthma as it is a variable disease. A diag-nosis of asthma should be based on history, physicalexamination, and the presence of airway obstructionin spirometry. In asthmatic children on treatmentBDR of > 10% was significantly associated with poorasthma control even when the pre-bronchodilatorspirometry was normal which suggests that BDR maybe a useful objective tool to assess asthma control inchildren.26

Severity grading for asthma

Along with other clinical characteristics, FEV1has been used to classify the severity of persistentasthma in children with mild, moderate, and severepersistent asthma having FEV1 > 80%, >60% to <80%,and ≤ 60% of predicted, respectively.27 The spirometryresults have been correlated with asthma severity evenin preschool children.28

Spirometry for pre-operative evaluation

Pulmonary function testing is not routinely in-dicated for pre-operative evaluation. It should be per-formed in children requiring lung resection surgery orin surgery other than lung resection (particularly tho-racic or upper gastro intestinal surgery) only if thereare clinical or radiological findings to suggest pulmo-nary abnormality.

Conclusion

Spirometry is the most widely performedpulmonary function test in adults but very much under-utilized in the paediatric age group as investigationaltool. It is a very useful investigation for evaluation andfollow-up of paediatric respiratory diseases. With bet-ter understanding of the respiratory physiology in chil-dren, availability of newer spirometry equipment andupdated reference values for the paediatric age group,spirometry is very much feasible in preschool groupalso. There is a need to encourage its use by primarycare physicians as well as paediatricians treating chil-dren with pulmonary diseases after adequate training.Its prompt use will certainly improve the standard ofcare of common paediatric pulmonary problems espe-cially asthma.

References

1. Kotecha SJ, Watkins WJ, Paranjothy S, Dunstan FD,Henderson AJ, Kotecha S. Effect of late pretermbirth on longitudinal lung spirometry in school agechildren andadolescents. Thorax 2012;67(1):54-61.

2. Chhabra SK, Vijayan VK, Rahman M, Mittal V, SinghPD. Regression equations for spirometry in childrenaged 6 to 17 years in Delhi region. Indian J ChestDis Allied Sci2012;54(1):59-63.


3. Dombkowski KJ, Hassan F, Wasilevich EA, ClarkSJ. Spirometry use among pediatric primary carephysicians. Pediatrics 2010;126(4):682-7

4. Veras TN, Pinto LA. Feasibility of spirometry inpreschool children. J Bras Pneumol2011;37(1):69-74.

5. Levy ML, Quanjer PH, Booker R, Cooper BG, HolmesS, Small I; General Practice Airways Group.Diagnostic spirometry in primary care: proposedstandards for general practice compliant withAmerican Thoracic Society and EuropeanRespiratory Society recommendations: a GeneralPractice Airways Group (GPIAG) document inassociation with the Association for RespiratoryTechnology & Physiology (ARTP) and Education forHealth. Prim Care Respir J 2009;

6. Miller MR, Hankinson J, Brusasco V, et al. ATS/ERSTask Force. Standardisation of spirometry.EurRespir J 2005;26(2):319-38.

7. Constant C, Sampaio I, Negreiro F, et al.Respiratory disease screening in school-agedchildren using portable spirometry. J Pediatr (RioJ) 2011;87(2):123-30.

8. Holt EW, Tan J, Hosgood HD. The impact ofspirometry on pediatric asthma diagnosis andtreatment. J Asthma 2006;43(7):489-93.

9. Vilozni D, Bentur L, Efrati O, et al. Spirometry inearly childhood in cystic fibrosis patients. Chest2007;131(2):356-61.

10. Chong HS, Moon ES, Park JO, et al. Value ofpreoperative pulmonary function test in flaccidneuromuscular scoliosis surgery. Spine2011;36(21):E1391-4.

11. Patra, K.P. (2012). Focus on diagnosis: Spirometry.Pediatrics in Review, 33(10), 469–472

12. KR Jatetal. Spirometery in children Prim CareRespir J 2013; 22(2): 221-229

13. Vilozni D, Barker M, Jellouschek H, Heimann G, BlauH. An interactive computeranimatedsystem(SpiroGame) facilitates spirometry in preschool


children. Am JRespirCrit Care Med 2001;164:2200-05.

14. Chavasse R, Johnson P, Francis J, Balfour-Lynn I,Rosenthal M, Bush A. To clip or not to clip? Noseclipsfor spirometry. EurRespir J 2003;21(5):876-8.

15. Beydon N, Davis SD, Lombardi E, et al. AmericanThoracic Society/European Respiratory SocietyWorking Group on Infant and Young ChildrenPulmonaryFunction Testing. An official AmericanThoracic Society/European Respiratory Societystatement: pulmonary function testing in preschoolchildren. Am J RespirCrit Care Med2007;175(12):1304-45.

16. Crenesse D, Berlioz M, Bourrier T, Albertini M.Spirometry in children aged 3 to 5 years: reliabilityof forced expiratory maneuvers. Pediatr Pulmonol2001;32:56-61.

17. Pistelli F, Bottai M, Carrozzi L, et al. Referenceequations for spirometry from a general populationsample in central Italy. Respir Med 2007;101(4):814-25.

18. Chhabra SK, Vijayan VK, Rahman M, Mittal V, SinghPD. Regression equations for spirometry in childrenaged 6 to 17 years in Delhi region. Indian J ChestDis Allied Sci2012;54(1):59-63.

19. Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF,Peslin R, Yernault JC. Lung volumes and forcedventilatory flows. Report Working PartyStandardization of Lung Function Tests, EuropeanCommunity for Steel and Coal. Official Statementof the European Respiratory Society. EurRespir J1993;16:5-40.

20. Barreiro TJ, Perillo I. An approach to interpretingspirometry. Am Fam Physician 2004;69(5):1107-14.

21. Glady CA, Aaron SD, Lunau M, Clinch J, Dales RE.A spirometry-based algorithm to direct lungfunction testing in the pulmonary functionlaboratory. Chest2003;123(6):1939-46.

22. Lebecque P, Kiakulanda P, Coates AL. Spirometryin the asthmatic child: is FEF25-75 a more sensitive



test than FEV1/FVC? PediatrPulmonol1993;16(1):19-22

23. Gelb AF, Zamel N. Simplified diagnosis of small-airway obstruction. N Engl J Med 1973;288:395-8

24. Pellegrino R, Viegi G, Brusasco V, et al.Interpretative strategies for lung functiontests.ATS/ERS Task Force Standardization of LungFunction Testing. EurRespir J 2005;26(5):948-68.

25. Global strategy for asthma management andprevention 2017 update chapter: Definitiondescription and diagnosis of asthma pg17

26. Galant SP, Morphew T, Newcomb RL, Hioe K, GuijonO, Liao O. The relationship of the bronchodilatorresponse phenotype to poor asthma control in

children with normal spirometry. J Pediatr 2011;158(6): 953-959.e1.

27. Stout JW, Visness CM, Enright P, et al. Classificationof asthma severity in children: the contribution ofpulmonary function testing. Arch PediatrAdolescMed2006;160(8):844-

28. Vilozni D, Barak A, Efrati O, et al. The role ofcomputer games in measuringspirometry inhealthy and ‘asthmatic’ preschool children. Chest2005;128:1146-55.

29. Aurora P, Stocks J, Oliver C, et al. Quality controlfor spirometry in preschool children with andwithout lung disease. Am J RespirCrit Care Med2004;169:1152-9.


Original Article

Pattern of Interstitial Pneumonitis inPatients with Systemic Sclerosis

Harsha P.V.*, D. Paul**, Jijith Krishnan***, Muraly C.P.*****Senior Resident, **Professor Pulmonary Medicine, *** Associate Professor General Medicine,**** Assistant Professor Pulmonary Medicine, Govt.Medical College, Thrissur

Abstract

Systemic Sclerosis (SSc) affects almost all parts of respiratory tract. Among the various pulmonarymanifestations the most common is the Interstitial Pneumonitis (IP) followed by pulmonary arterial hyperten-sion (PAH). Early identification of IP is important as it has prognostic implications. Most studies describe NonSpecific Interstitial Pneumonitis (NSIP) as the predominant pattern of IP. This study is undertaken to explore thepossibility of a regional variation in the pattern of IP in systemic sclerosis.

Methods

This is a Cross sectional Observational study in consecutive patients satisfying American College ofRheumatology criteria for SSc and having lung involvement either clinically or by investigations. They wereevaluated with high resolution computed tomography (HRCT) and the pattern of lP was assessed.

Results

A total of 30 patients were enrolled in the study. Dyspnoea on exertion was present in 26 (86.6%)patients. 7 (23.3%) patients had cough at presentation, and 8 (26.6%) had chest pain. HRCT was suggestive of IPin 22 (73.3%, 95% CI 55.6 -85.8%) patients. Among the IPs the most common was Usual Interstitial Pneumonitis(UIP) 13 (59%, 95% CI 38.7-76.7%). Non Specific Interstitial Pneumonitis (NSIP) pattern was present in only 6(27.2%, 95% CI 13.2-48.2%) and 3 (13.6%, 95% CI 4.7-33.3%) had mosaic pattern. Mean Forced Vital Capacity(FVC) was 66.7%. Mean Forced Expiratory Volume in the first second (FEV1) was 67.5%.The mean 6 Minute WalkDistance (6MWD) was 408.9±74.19m. Significant exercise desaturation of more than 4% in 6MWT was found in3(10%) patients and all were having UIP pattern.The sensitivity of exercise desaturation testing to identify IP inSSc was only 14.3%.

Conclusion

There was high prevalence of IP in patients with SSc and the most predominant pattern of IP was UIP.Exercise induced saturation fall was not a sensitive tool in identifying patients with IP.

Key words

Systemic Sclerosis, Interstitial pneumonias


Introduction

Systemic Sclerosis (SSc) is an autoimmunedisease with functional and structural abnormality insmall blood vessels along with interstitial fibrosisaffecting mainly the skin and internal organs. SystemicSclerosis affects almost all aspects of respiratory tractincluding blood vessel, airways, pleura, parenchymaand musculature. Interstitial Lung Disease (ILD) andPulmonary Arterial Hypertension (PAH) are thepredominant manifestations; others being aspirationpneumonitis, obliterative bronchiolitis, pleural reactions,restrictive ventilatory defect due to chest wall fibrosis,spontaneous pneumothorax, lung cancer, particularlybronchoalveolar carcinoma and pulmonaryhemorrhage due to endobronchial telangiectasia. Lunginvolvement is found at autopsy in 70 to 100% of patientswith systemic sclerosis (SSc)1.

Lung manifestations usually follow systemicfeatures in Systemic Sclerosis. But reports of respiratorysymptoms presenting before the developmentof the skin lesions have also been reported. Antitopoisomerase2,3 and antiendothelial cell4 antibodiespredict the presence of lung involvement, whileanticentromere and anti RNA polymerase III antibodiesare less associated with lung disease.

Limited cutaneous SSc has a relatively goodprognosis with a long-lasting disease duration and the10-year survival rate is about 80 to 90%. However,presence of pulmonary arterial hypertension which isseen in about 10% of these cases may lead to a moresevere prognosis5. A study done on Canadianscleroderma (SSc) patients to assess the morbidity andmortality concluded that cardiac involvement, diffusecutaneous SSc, and hypertension are associated withworse survival.

A rapid decline in Diffusing Capacity for carbonmonoxide (DLCO) or lung volumes may predict poorsurvival. The overall mortality rate in scleroderma is50% at 7 years, and pulmonary complications are themajor cause of death. But survival of patients withscleroderma is improving compared with older reportsin the literature.

Aim

To find out the prevalence and pattern of IP inpatients with SSc attending the Rheumatology clinic ofour institute.

Materials and methods

Study Design :

This was a Cross sectional Observational study.

Study population :

This study was conducted in 30 patientsattending the Rheumatology clinic satisfying AmericanCollege of Rheumatology criteria for SSc and havingany pulmonary symptom or radiographic or PFTabnormality or any combination of these. Patients withsputum Acid Fast Bacilli (AFB) positivity and cardiacfailure were excluded.

Methods

Patients included in the study were interviewedpersonally. Detailed history including sociodemographicinformation, clinical symptoms, co morbidities, smokingstatus and clinical signs were recorded. Routineinvestigations were done in all patients includingsputum for AFB. 6MWT was done for all patients whosesaturation was >88%. 6MWD was recorded and oxygensaturation was recorded using pulse oximeter pre andpost exercise. Chest X-ray and ECG was taken and PFTwas done to know whether restrictive or obstructivepattern. HRCT was done in all patients and findingswere recorded. Data was tabulated and prevalenceand proportions of HRCT patterns were studied andcorrelated clinically.

Results

A total of 30 patients were enrolled in thestudy.All were females.The mean age was 43.03 ± 7.47years. Mean duration of the disease at presentationwas 2.3 ± 3.6 years with a maximum of 15 years and aminimum of 1 month.

Harsha P.V. - Pattern of Interstitial Pneumonitis in Patients with Systemic Sclerosis



Fig 1 :Distribution of subjects according to age

Dyspnoea on exertion was present in 26 pa-tients (86.6%). 7 (23.3%) patients had cough at presenta-tion, 8 (26.6%) patients had chest pain. 18 (60%) patientsalso complained of fatiguability. Pallor and edema werefound in 2 (6%) patients. Clubbing and cyanosis was foundin 1 (3.3%) patient. 16 (53.3%) patients had crepitationssuggestive of ILD. Chest x ray was abnormal in 16 (53.3%)patients out of these, 14 (46.6%) had IP pattern. ECGshowed p pulmonale in 3(10%) patients.

Fig 2 :Frequency of systemic features

Spirometry showed restrictive abnormality in22 (73.3% 95% CI 55.6-85.8% ) patients and was normalin rest of the patients. Among IP patients,FVC was re-duced in 19 patients and was normal in 3 patients. MeanFVC was 66.7±16.4%.Mean FEV1 was 67.5 ±15.3%. Pa-tients with IP showed statistically significant reductionin FVC compared to non IP patients. (p0.01). There wasno statistically significant reduction in FVC in UIP (p0.05) or NSIP (p0.5) when taken alone. 6MWD was mea-sured in all patients with resting saturation >88%. Themean 6MWD was 408.9±74.19 metres. Compared to non-

Table 1Comparison of FVC among Interstitial Pneumonitis (IP)

and non Interstitial Pneumonitis (non IP) patients

IP non IP p value

Mean FVC% 61.9 79.75 0.01

Arterial hypoxemia (<92%) at rest was found in2 patients. Patient who were unable to perform 6MWTshowed significant exercise desaturation even with mildexertion like standing from lying down position. Signifi-cant exercise desaturation of more than 4% in 6MWTwas found in 3 (10%) patients and all were having UIPpattern. The sensitivity of exercise desaturation testingto identify IP in SSc was only 14.3%. There was nostatistically significant difference between pre andpost exercise saturation among IP (p0.08), UIP(p0.07) orNSIP.

ANA profile was done for 22 patients.12 patientshad antitopoisomerase Ab positive and 6 patients hadanticentromere Ab positive ,4 patients had a negativeANA profile . Other Ab found were SS-A , SS-B,Ro-52,RNP/Sm, Jo-1.

HRCT was abnormal in 28 patients and wasnormal in rest.On HRCT, 18(60.0%) had reticular le-sions,11(36.6%) had ground glassing (out of 11,2 had onlyminimal ground glassing), 10(33.3%) had honeycomb-ing, 8(26.6%) had traction bronchiectasis, Fibrosis with /without traction bronchiectasis alone was found in 3(10%)patients.23(76.67%) patients had bilateral lesions andsubpleural lesions were found in 13(43.33%)patients.Lesions showed a lower lobe predliction in23(76.67%) patients. Localized ground glassing was foundin 2 patients. Alveolar opacities and centrilobular nod-ule was found in 1 patient each. With HRCT, IP was diag-nosed in 22(73.3%) patients. Out of the IP patients,13(59%95% CI 38.7-76.7%) had UIP pattern, 6(27.2% 95% CI 13.2-48.2%) had NSIP pattern,3(13.6% 95% CI 4.7-33.3%) hadmosaic pattern.

UIP patients, UIP patients had statistically significantreduction in the mean 6MWD (p0.02), there was no sig-nificant difference between IP and non-IP (p 0.12) orNSIP and non NSIP patients.



Fig 3 : Prevalence of IP (Interstitial Pneumonitis)in Systemic Sclerosis

Fig 4 : HRCT findings

Fig 5 :Distribution of patterns of IP in Systemic Sclerosis

Discussion

Systemic Sclerosis is an autoimmune diseasewith a high prevalence of Interstitial Pneumonitis. Ingeneral, there is a greater prevalence of autoimmunedisease in females and systemic sclerosis is no excep-tion. The disease is more commonly diagnosed in thethird and fourth decades, and most patients in this studywere in the middle age group with mean age of 43.03years.

Patients with interstitial Pneumonitis usuallypresent with shortness of breath and dry cough. In ourstudy, dyspnoea on exertion was the most common res-piratory complaint while some patients also complainedof chest pain and dry cough. These results are similar toprevious Indian studies.

Among systemic symptoms, sclerodactyly wasseen in all patients. Joint symptoms, raynauds phenom-enon, pigmentation and pitting scars were common andwas found in 66.6%, 83.3%, 53.3% and 56.6% respectively.The degree of skin thickening depends on the subtypeand duration of disease while cold-induced Raynaudsphenomenon has been mentioned the most commonmanifestation of systemic sclerosis, occurring in morethan 95 percent of patients, according to previous lit-erature. Clubbing, cyanosis and edema were rare. ChestX-Ray could detect only 14 cases of IP. With conven-tional radiography, prevalence of IP in this study wouldhave been 46.6% and with HRCT it was 73.3%. HRCT hasdefinitely increased the sensitivity for detection ofILD6,7,8. Various literatures have shown sensitivity rang-ing from 70% to 90% for detecting ILD with HRCT.

Exercise desaturation is a classic finding in Id-iopathic Pulmonary Fibrosis (IPF). Superiority of exer-cise testing over resting pulmonary function tests indetermining the nature and extent of physiologic de-rangements in ILD have been demonstrated in severalstudies. In one study by Jean etal, consisting of 15 sys-temic sclerosis patients, arterial hypoxemia (below 95per cent) was found in only three cases at rest and inseven on mild exercise9. In a study by Wander et al, on6MWT in Scleroderma patients reported exercisedesaturation in 28%10. In Fujiko et al’s study of Sclero-derma patients, exercise desaturation was found in37%11. In this study, significant exercise desaturationwas found only in 3 (10%) patients and there was no


statistically significant difference between pre and postexercise saturation among IP, UIP or NSIP.

Compared to non-UIP patients,UIP patientshad statistically significant reduction in 6MWD whilethere was no significant difference between IP and non-IP or NSIP and non-NSIP patients. This was similar toWander etal’sstudy “Six-Minute Walk Test for the Evalu-ation of Pulmonary Disease Severity in SclerodermaPatients” where 6MWD decreased with increase in lungfibrosis.

Baseline gas transfer DLCO and FVC levelsare measures of progression of the disease severityand reductions in both parameters have been associ-ated with increased mortality in IP. Jonathan etal’s studyon HRCT findings reported that pulmonary fibrosis wassignificantly negatively correlated with FVC, whileground glass abnormalities did not correlate well withFVC12. In this study FVC was reduced in 19 InterstitialPneumonitis patients and was normal in 3 IP patients.With spirometry alone, the prevalence of IP would havebeen 63.3%. However, the wide range of normal FVC(80–120% of predicted) is a major constraint. For ex-ample, a mildly reduced FVC level of 75% represents aclinically insignificant reduction from a premorbid FVClevel of 80% but a striking decline from a premorbidvalue of 120%. Thus, single FVC levels are likely to bemisleading when disease is either minimal or severe13

but serial FVC levels are definitely a simpler,safer andcost effective method to monitor the progression of dis-ease.

Though lung biopsy is the gold standard fordiagnosis of ILD, HRCT is now the investigation of choicein ILD. In HRCT, literature says reticular and groundglassing as the common patterns in SSc patients. Sev-eral studies reported ground glassing as the most com-mon pattern in SSc patients, while our study showedreticular lesions as the most common followed byground glassing and traction bronchiectasis. In the SLS(Scleroderma Lung Study), pulmonary fibrosis andground glassing were the most common HRCT scanabnormalities. In our study, honey combing was seen in33.3% which is similar to the finding in the study by Remy-Jardin et al (35.8%; 19 of 53 patients)14, but is higher thanthat in the study by Akira et al (11%; 1 of 9 patients)15.UIP was more prevalent than NSIP in the present study.Almost all the previous studies have reported NSIP as

the common pattern in HRCT. It was found in SLS thatthe extent of pulmonary fibrosis seen on baseline HRCTscans was predictive of the progression rate in the ab-sence of immunosuppressive therapy. HRCT providesexcellent parenchymal detail and has been widely ac-cepted as the reference technique for noninvasive di-agnosis of interstitial lung disease. Various studies haveshown that in systemic sclerosis patients with lung in-volvement, HRCT abnormalities correlate well with lunghistology. HRCT can also be used to predict the progno-sis of lung involvement.

Conclusion

There was high prevalence of IP in patientswith SSc and the most predominant pattern was UIP.Exercise induced saturation fall was not a sensitive toolin identifying patients with IP.

Limitations

There were only limited numbers of patients.Another major limitation was the absence of open lungbiopsy which is the gold standard for the diagnosis of IP.In this study ECHO test for assessing PAH and DLCOfor assessing diffusion capacity was also not included.

References

1. D’Angelo WA, Fries JF, Masi. AT, Shulman LE.Pathologic observations in systemic sclerosis(scleroderma). Am J Med. 1969;46(3):428–40

2. Greidinger EL, Flaherty KT, White B, Rosen A,Wigley FM, Wise RA. African-American race andantibodies to topoisomerase I are associated withincreased severity of scleroderma lung disease.Chest. 1998;114(3):801–7.

3. Steele R, Hudson M, Lo E, Baron M. Clinical decisionrule to predict the presence of interstitial lungdisease in systemic sclerosis. Arthritis Care Res(Hoboken). 2012;64(4):519–24.

4. Lewandowska K, Ciurzynski M, Gorska E, Bienias P,Irzyk K, Siwicka M, et al. Antiendothelial cellsantibodies in patients with systemic sclerosis inrelation to pulmonary hypertension and lungfibrosis. AdvExp Med Biol. 2013;756:147–53.




5. Sampaio-Barros PD, Zimmermann AF, Müller CdeS, Borges CT, Freire EA, Maretti GB, Marques NetoJF, Salgado MC, SaumaMde F, de Azevedo MN,Fontenelle S, Kayser C; Systemic SclerosisCommission of the Brazilian Society ofRheumatology. Recommendations for themanagement and treatment of systemic sclerosis.Rev Bras Reumatol. 2013 May-Jun;53(3):258-75.

6. Warrick JH, Bhalla M, Schabel SI, Silver RM. Highresolution computed tomography in earlyscleroderma lung disease. J Rheumatol .1991;18(10):1520–8.

7. Spillane RM, Shepard J a O, Deluca S a. High-resolution CT of the lungs. Am Fam Physician.1993;48(3):493–8.

8. Collins CD, Wells AU, Hansell DM, Morgan RA,MacSweeney JE, du Bois RM, et al. Observervariation in pattern type and extent of disease infibrosingalveolitis on thin section computedtomography and chest radiography. ClinRadiol.1994;49(4):236–40.

9. Ashba JK. The Lungs in Systemic Sclerosis. CHESTJ . 1965;47(1):52.

10. WO Villalbaetal. Six - Minute Walk Test for the

Evaluation of Pulmonary Disease Severity inScleroderma Patients CHEST 2007; 131:217–222)

11. Someya F. Predictors of Exercise - Induced OxygenDesaturation in Systemic Sclerosis Patients WithInterstitial Lung Disease. 2015;4187.

12. Goldin JG. High-Resolution CT Scan Findings inPatients With Symptomatic Scleroderma-RelatedInterstitial Lung Disease<xref rid=“AFF1”>*</xref>.CHEST J. 2008;134(2):358.

13. Goh NSL, Desai SR, Veeraraghavan S, Hansell DM,Copley SJ, Maher TM, et al. Interstitial LungDisease in Systemic Sclerosis. Am J RespirCrit CareMed. 2008;177(11):1248–54.

14. Remy-Jardin M, Remy J, Wallaert B, Bataille D,Hatron PY. Pulmonary involvement in progressivesystemic sclerosis: sequential evaluation with CT,pulmonary function tests, and bronchoalveolarlavage. Radiology. 1993;188(2):499–506.

15. Akira M, Inoue G, Yamamoto S, Sakatani M. Non-specific interstitial pneumonia: findings onsequential CT scans of nine patients. Thorax.2000;55(10):854–9.


Radiology Quiz

Vishnu Sharma M.Professor and Head, Dept of Respiratory Medicine,A J Institute of Medical Sciences, Mangalore, Karnataka

Corresponding Author : Vishnu Sharma M.

Professor and Head, Dept of Respiratory Medicine,A J Institute of Medical SciencesMangalore, Karnataka, PIN 575 004Phone : 0824 2216321, Mobile: 09448126321Email: [email protected]

Question : What is the most likely diagnosis?

Fig 1 Fig 2


ANSWER

Chest x-ray PA and lateral views show multiplenodular opacities over the lung fields, scapula and softtissue over the chest and abdomen. Fibrotic changesare seen in the right upper zone. Since the nodular opaci-ties are seen over the soft tissues of chest and abdo-men a diagnosis is most likely to be multiple soft tissueswellings. The most common condition leading to mul-tiple soft tissue swellings in the body is neurofibromato-sis type one1. Physical examination of the patient con-firmed the diagnosis of neurofibromatosis type one.

This patient was referred from department oforthopaedics for evaluation of abnormal chest radiog-raphy. Radiologist had reported the chest x ray as mul-tiple lung secondaries. He was a 55 year old male ad-mitted for evaluation of low back pain. On enquiry hehad no history of breathlessness, chest pain, hemopty-sis, and fever or weight loss. Apart from low back painhe had no other symptoms. He had pulmonary tubercu-losis 15 years back for which he took regular Anti Tuber-cular Treatment.

Chest X-ray PA view was taken which showedbilateral multiple opacities which was reported as mul-tiple lung secondaries by radiologist. Careful history,physical examination and lateral view confirmed thediagnosis as neurofibromatosis type 1.

Soft tissue lesion in the chest can lead to ab-normal chest x ray. Careful physical examination andlateral view will help in identifying the cause for radio-logical abnormality2. Lack of awareness about soft tis-sue lesions leading to radiological abnormalities will leadto unnecessary further investigations.

An opacity seen in the chest x ray can be dueto pathologies in the skin, subcutaneous tissue, muscles,

bone, lungs, heart and mediastinum3. Lateral film willhelp to localize the opacity. The easiest way to locatesoft tissue swellings is by clinical examination. Ideallychest x rays should be interpreted correlating with his-tory and clinical examination.

Some causes for nodular shadows in bilaterallung fields are Metastasis, Granulomas, Hydatid dis-ease, Wegener’s granulomatosis and Arterio venousmalformations1.

Importance of looking at soft tissue inchest x ray

Soft tissues can cast abnormal shadow in chestx-ray which may be misinterpreted as lung lesion if clini-cal examination findings and lateral view is not takeninto account during interpretation. Absence of soft tis-sue on one hemi thorax may lead to hyper translucencyon the same side, which may be mistaken for pneu-mothorax or bulla.

References

1. Parichehr Ghalayani, Zahra Saberi and FarimahSardari Neurofibromatosis type I (vonRecklinghausen's disease): A family case report andliterature review. Dent Res J (Isfahan), 2012 Jul-Aug;9(4): 483–488.

2. Se Jin Nam, MD, Sungjun Kim, MD, Beom Jin Lim,MD et al,,Imaging of Primary Chest Wall Tumorswith Radiologic-Pathologic Correlation.RadioGraphics 2011; 31:749–770

3. Hillier JC, Moskovic E, The soft-tissuemanifestations of neurofibromatosis type 1. ClinRadiol. 2005 Sep; 60(9):960-7

Vishnu Sharma M. - Radiology Quiz


Case Report

An Unusual Cause of Chest Pain

Arjun P.*, Abin Varghese Thomas**, Madhavan Unni****Senior Consultant and Head, **Registrar, Department of Respiratory Medicine,*** Senior Consultant & Head, Dept. of Radiodiagnosis,Kerala Institute of Medical Sciences, Trivandrum - 695029, India.

Introduction

Chest pain following bronchial arteryembolisation is a commonly observed transient adverseevent. However persistent chest pain is quite rare. Wepresent the case of a 26 year healthy male whounderwent bronchial artery embolisation for massivehemoptysis, but developed progressive and persistentchest pain following the procedure. On detailedevaluation, he was detected to have chest painattributable to focal ischemia due to non targetembolisation

The Case

A 26 year old otherwise healthy male, acutelydeveloped six bouts of massive hemoptysis. He was asmoker (10 pack years) but denied having any pastrespiratory illness including tuberculosis. He was

admitted in a local hospital, where he was treated withcough suppressants and hemostatics. Subsequently heunderwent a flexible fibreoptic bronchoscopy (which didnot reveal any abnormality) and a HRCT of the Thorax,which showed the presence of alveolar opacities in theposterior segment of the right upper lobe. In view ofongoing bleed, he underwent bronchial arteryembolization with successful results. However, in theimmediate post- procedure period, he developed adiffuse chest pain mainly involving the right side. It wasa constant dull, aching pain, with no identifiableaggravating or relieving factors. A Chest X Ray wastaken which was normal. He also underwent a detailedcardiac assessment, which also did not reveal any causefor his pain. In this context, he was referred to us forfurther management.

Clinical examination of the respiratory systemwas normal. He was hemodynamically stable as well. A

Abstract

Bronchial artery embolization is commonly done to control bleeding, when patients present withmassive hemoptysis. In experienced hands, it is a relatively safe procedure. Adverse eventsfollowing bronchial artery embolization are usually transient. Herein we present an uncommonadverse event following bronchial artery embolization.

Keywords : Bronchial artery embolization, chest pain, non target embolization


Arjun P. - An Unusual Cause of Chest Pain

Fig 1 : Radioopaque embolic material (arrow) seenwithin the muscular branches of posterior intercostal

arteries

CT angiogram was taken which revealed the presenceof radiodense material inside the chest wall muscle (Fig.1arrow). An identical radiodense opacity abutting thepleural surface was also noted (Fig.2). These wereconsistent with the embolized glue getting depositedinside the muscular and subpleural branches of theintercosto-bronchial arteries, possibly causing focalischemia, which had led to development of the chestpain. He was started on analgesics and had significantpain relief thereafter.

Fig 2 : Radioopaque embolic material seen within thepleural branches of the intercoastal arteries

Discussion

Transient chest pain is the most common com-plication observed following bronchial arteryembolisation1. However the mechanism involved is notfully understood. Other complications of the procedure

include subintimal dissection of the aorta or bronchialartery, pulmonary infarction, bronchial cartilage necro-sis, transient vision loss, and spinal cord ischemia2. Dys-phagia following inadvertent embolization of the oe-sophageal branches has also been described. Our pa-tient had severe, persistent chest pain and we wantedto rule out major complications like subintimal dissec-tion, pulmonary infarction etc which lead us to do a re-peat CT angiogram.

Glue by itself is not radio opaque. To make itradio-opaque, glue is mixed with Lipiodol, an oil basedradio-opaque contrast medium before embolisation.Embolization material getting dislodged into the chestwall muscle has never been reported. Bronchial arter-ies are modified intercostal arteries. Some of them havemuscular branches, proximal to the origin of bronchialartery proper, to which non-target embolisation can oc-cur, when antegrade flow to bronchial artery is stoppedby embolic material or vasospasm

We speculate that it may be a common occur-rence which results in post procedure chest pain butremains under detected. With the increasing access ofsuperselective catheterisation with microcatheters andof emboliszation particles of appropriate sizes, non tar-get embolization should become quite rare3.

References

1. Srivastava DN, Manisha J, Bhalla A, Thulkar S,Sharma S. Bronchial Artery Embolization inPulmonary Diseases: Current Scenario. JIMSA.2013;26(1):69–71.

2. Lopez JK, Lee H-Y. Bronchial artery embolizationfor treatment of life-threatening hemoptysis. SeminIntervent Radiol. 2006 Sep;23(3):223–9.

3. Ingbar DH. Massive hemoptysis: initialmanagement. Available from url: http://www.uptodate.com. Accessed on Feb 18, 2016.


Case Report

A Case Report of Young’s Syndrome and Review of Literature

Venu Gopal Panicker*, Arvind Kumar Misra**, Shajahan Purathel Sulaiman****Additional Professor, **Senior Resident, ***Associate ProfessorDept. of Pulmonary Medicine, Government TD Medical College, Alappuzha

Introduction

Young’s syndrome is a rare clinical scenario,usually presenting with chronic sino - pulmonarysymptoms due to bronchiectasis and infertility due toobstructive azoospermia. It is a diagnosis of exclusionby excluding commoner causes like cystic fibrosis andprimary ciliary dyskinesia. It is of unknown etiology andmay present in families.

Case report

A 23-year-old non smoker presented withchronic cough with purulent sputum since earlychildhood. He had occasional episodes of minorhemoptysis in last year. He also had history suggestiveof chronic sinusitis. He had clubbing. Respiratory systemexamination demonstrated bilateral medium rales, morein the lower areas. The patient was unmarried. He gavea positive family history of chronic respiratorysymptoms and infertility in his cousin brother andmaternal uncle.

Radiographic Findings

A chest radiograph and HRCT images of thechest shows evidence of bilateral cylindricalbronchiectasis with secondary infection.( Fig 1 A, 1B and1C). There was no dextrocardia.

Abstract

We present a young male with bilateral bronchiectasis, chronic sinusitis and azoospermia. Thereare no clinical or laboratory evidence of alternative diagnosis of cystic fibrosis and primaryciliary dyskinesia. The case is being presented due to its rarity.

Key words : Bronchiectasis, Azoospermia, Young’s syndrome.

Fig : 1 A


Venu Gopal Panicker - A Case Report of Young’s Syndrome and Review of Literature

Laboratory Findings

Routine blood results were normal. Pulmonaryfunction test results showed mild restriction. Serum to-tal IgE was 54 IU/ml (The normal value of IgE in adult isless than 158 IU/ml) and Aspergillus specific IgE wasnegative, which rules out allergic bronchopulmonaryaspergillosis. Diffusion capacity of the lung for carbonmonoxide was normal. Ultrasound scan did not revealany situs inversus. Sweat chloride test was 24 mmol/L(normal limit is 0 - 39 mmol/L).

Saccharin test time was 54 minutes which dem-onstrated a delayed mucociliary transport time (nor-mal 10-20 minutes).

He was referred to a Urologist. Semen analy-sis repeatedly revealed absolute azoospermia whichsuggested a diagnosis of Young’s syndrome. Physicalexamination of the external genitalia was essentiallynormal. Ultrasound scan examination of the testes, vasde ferns and the epididymis were normal.

Diagnosis:

A triad of obstructive azoospermia, chronicrhino sinus disease and bronchiectasis favors a diagno-sis of Young’s syndrome. There are no other features ofcystic fibrosis and the sweat chloride test was normal.Primary ciliary dyskinesia is characterized by immotilesperms, not azoospermia. But the patient was unwillingfor a testicular biopsy which would have confirmed thediagnosis.

Review of Literature

The term Young’s syndrome was coined by aLiverpool Urologist David Young in 19701. In 1978 Hendryclassically described this syndrome as a complex of ob-structive azoospermia, sinusitis and bronchiectasis.Usually this condition affects young males. Symptomspertaining to sinuses usually disappear during adoles-cence. Pulmonary symptoms and obstructive azoosper-mia usually persist. These patients usually seek medi-cal help for infertility rather than for sino pulmonarysymptoms.

Even though the exact cause of Young’s syn-drome is unknown, exposure to mercury has been at-

Fig : 1 B

Fig : 1 C

CECT of paranasal sinuses showed polypoidalmucosal thickening of bilateral ethmoid, maxillary andsphenoid sinuses, suggestive of chronic sinusitis. (Fig 2)

Fig : 2


tributed to be the reason behind this occurrence. Defi-ciency of an enzyme protein- carboxyl methylase hasbeen reported in such patients2. These enzymes arenecessary for normal glycolysis. Motility of sperm ishighly dependent on glycolysis and hence is affected inthese patients. Similarly motility of cilia is dependenton the energy released due to glycolysis. With reduc-tion of mercury usage the incidence of Young’s syn-drome has come down drastically. However, our pa-tient did not reveal any history of mercury exposure.

Prevalence is unknown due to under reporting,but thought to be much less than primary ciliary dyski-nesia. Young’s syndrome is an autosomal recessivedisorder and has been identified in identical twins.Offending gene causing this problem has not yet beenidentified.

This condition typically affects young males.Symptoms pertaining to sinuses usually disappear dur-ing adolescence. Pulmonary symptoms and obstruc-tive azoospermia usually persist.

There is no Specific diagnostic test for Young’ssyndrome. The diagnosis is usually by the process ofexclusion, especially cystic fibrosis and primary ciliarydyskinesia3. All patients with infertility and sinus disor-ders should be evaluated. A prolonged saccharin testindicates impaired mucociliary clearance, which is de-layed in primary ciliary dyskinesia also. Unlike in pri-mary ciliary dyskinesia, electron microscopy study ofnasal mucosa is usually normal in Young’s syndrome4.

Even though the patients exhibit normal sexualdevelopment, libido and potency, they are usually in-fertile. Young's syndrome is characterized by inspis-sated secretions within the vas and epididymis whichinterfere with transport of sperm, leading to obstruc-tive azoospermia5. Semen analysis usually demon-strates complete azoospermia. Epididymis may be en-larged or cystic, with lots of spermatozoa. A testicularbiopsy will show normal spermatogenesis.

Chest Xray and HRCT usually reveals bron-chiectasis. Xray and CECT of paranasal sinuses mayshow mucosal hypertrophy and evidence of chronicsinusistis.

It has been postulated that Young’s syndrome

is a variant of primary ciliary dyskinesia. Even thoughdextrocardia and situs inversus are part of the triad ofKartagener’s syndrome, there have been reports ofYoung’s syndrome having these features6.

Treatment involves management of bron-chiectasis with appropriate antibiotics, chest physio-therapy, mucolytics, etc. With the help of microsurgicaltechniques and treatment modalities like intracytoplas-mic sperm injection, infertility can be managed.

Acknowledgements

Dr. Thomas Daniel, MS, MCH, MRCS. Consult-ant Urosurgeon, General Hospital, Alappuzha.

References

1. Young's Syndrome - Obstructive Azoospermia andChronic Sinopulmonary Infections David J.Handelsman, Ann J. Conway, Lyn M. Boylan, andJohn R. Turtle. N Engl J Med 1984; 310:3-9January 5,1984

2. Claude Gagnon, Richard J. Sherins, David M. Phillipsand C. Wayne Bardin. Deficiency of Protein-Carboxyl Methylase in Immotile Spermatozoa ofInfertile Men. N Engl J Med 1982; 306:821-825 April 8,1982.

3. Ichioka K, Kohei N, Okubo K, Nishiyama H, Terai A.Obstructive azoospermia associated with chronicsinopulmonary infection and situs inversus totalis.Urology. 2006;68(1):204.e5.

4. Michael R. Knowles , Leigh Anne Daniels , StephanieD. Davis , Maimoona A. Zariwala , and Margaret W.Leigh Primary Ciliary Dyskinesia. Recent Advancesin Diagnostics, Genetics, and Characterization ofClinical Disease. AJRCCM. Vol. 188, No. 8 | Oct 15,2013 PubMed: 23796196

5. Fertility in men with primary ciliary dyskinesiapresenting with respiratory infection. Munro NC,Currie DC, Lindsay KS, Ryder TA, Rutman A, DewarA, Greenstone MA, Hendry WF, Cole PJ Thorax.1994;49(7):68

6. Young's syndrome (obstructive azoospermia andchronic sinobronchial infection): a quantitative studyof axonemal ultrastructure and function. Wilton LJ,Teichtahl H, Temple-Smith PD, Johnson JL,Southwick GJ, Burger HG, de Kretser DM Fertil Steril.1991;55(1):144.

Venu Gopal Panicker - A Case Report of Young’s Syndrome and Review of Literature


Case Report

Lung Abscess Mimic- A Case Report

Ravindran Chetambath*, Jabeed Parengal**,Mohammed Aslam***, Sanjeev Shivashankaran****Professor & Head, ** Assistant Professor, *** Senior ResidentDept. of Pulmonary MedicineDM Wayanad Institute of Medical SciencesWayanad, Kerala

Corresponding Author :Dr. Ravindran Chetambath, Professor & HeadDept. of Pulmonary MedicineDM Wayanad Institute of Medical SciencesWayanad, Kerala, India

Introduction

Chest physicians generally acknowledge a linkbetween esophageal disorders and respiratorydiseases. This relationship is however complex and maymanifest in a variety of clinical entities. There are fewstructural abnormalities of esophagus such asesophageal achalasia, esophageal diverticulum,esophageal obstruction, and tracheo-esophageal fistula(congenital or acquired), which can lead on to recurrentaspiration and devastating respiratory problems.Radiologically esophageal diverticulum can simulate a

lung abscess by its position and appearance with airfluid level. This may be usually misdiagnosed as lungabscess, as the patient may have respiratory symptomsdue to regurgitation and aspiration. It is important forthe pulmonary physician to consider the diagnosis ofesophageal abnormality in patients with anorexia, chestpain, dysphagia, regurgitation, weight loss or chroniccough especially after food intake. Often thesesymptoms are attributed to less serious pathology,leading to delay in diagnosis of structural diseases ofesophagus.

Abstract

Esophageal diverticula are congenital or acquired out pouching of esophagus which retain foodand cause regurgitation and aspiration. Because of its peculiar location and radiological appear-ance simulating a cavity with fluid level, it is often mistaken for lung abscess. Here we report acase being referred as lung abscess to our outpatient department which on investigation turnedout to be esophageal diverticula.

Key Words : Lung Abscess, Esophageal Diverticulum, Aspiration Pneumonia


Case Report

This is the case history of a 63 year old femaleschool teacher presenting with fever, body ache andcough for 2 days. She was treated with antibiotic asoutpatient by her family physician. Since there was noclinical improvement on the second day, an X-ray chestwas ordered and case was referred as Lung abscess.Clinically patient was febrile with profuse crackles onthe right side. Her vital status was stable. Her historyrevealed a medical evaluation including upper GIendoscopy 2 years back. She was advised only periodicfollow up. X ray chest of the patient on admission showedan opacity right lower zone with air fluid level suggestiveof lung abscess (Fig-1). She was admitted as a case ofaspiration pneumonia and lung abscess and put onantibiotics covering anaerobic organism. She wasworked up for causes of aspiration. Routine bloodexamination was normal. Her random blood sugar was184 mg% and blood urea was 18mg%. Sputum culturewas sterile. CT Thorax showed a large diverticulum onthe lower third of esophagus towards the right side (Fig-2). X ray taken after nasogastric tube aspiration of foodmaterial from the diverticulum showed completedisappearance of the earlier shadow (Fig-3). A bariumcontrast esophagogram demonstrated the out pouchingof lower end of esophagus towards right side (Fig-4).Upper GI endoscopy revealed large diverticular sac (Fig-5). She was referred to surgical gastroenterologist forsurgical repair of diverticulum.

Fig 1 : X-Ray chest showing opacity Right lower Zonewith air fluid level.

Fig 2 : CT Thorax showing esophageal pouchtowards right side.

Fig 3 : X-Ray Chest PA after nasogastric aspiration.The lower zone shadow is not seen.

Fig 4 : Ba contrast X-ray demonstratingesophageal diverticulum.

Ravindran Chetambath - Lung Abscess Mimic- A Case Report


Fig 5 : Esophagoscopy demonstratingright sided diverticulum.

Discussion

Esophageal diverticulum is an out-pouching orsac of the epithelial-lined tissue of the esophagus. Itmay be a true diverticulum, involving all layers of theesophagus, or false diverticula, involving only the mu-cosal and sub mucosal layers that protrude into the cir-cular and longitudinal muscle of the esophagus1.

Diverticulum is generally classified by esoph-ageal location such as upper (pharyngo-esophageal orZenker Diverticulum), middle, or lower (epiphrenic)1.Further categorization relates to presumed etiology, thatis either traction or pulsion. Mid-esophageal diverticularesult from traction as a consequence of long-standingmediastinal inflammation or fibrosis - as in tuberculosisor histoplasmosis1. Pharyngoesophageal and epiphrenicdiverticula usually result from an esophageal motilitydisorder leading to repeated increased pressure in thearea of the diverticulum. Epiphrenic diverticula typicallyoccur within 10 cm of the esophago-gastric junction2.Diverticula may cause anorexia, chest pain, dysphagia,epigastric pain, halitosis, heartburn, nocturnal cough,odynophagia, regurgitation, or weight loss2. However,in as many as 40% of patients, the diverticulum will beasymptomatic3. Esophagogastroduodenoscopy is rec-ommended for the detection of these pathology1. A de-lay in the diagnosis and treatment of epiphrenic diver-ticula can lead to severe complications. Patients are atrisk of regurgitation and gastrointestinal bleeding1. Re-tention of undigested food in large diverticula occasion-ally results in regurgitation, nocturnal cough, aspiration

Ravindran Chetambath - Lung Abscess Mimic- A Case Report

pneumonia and lung abscess4. Very severe, persistentaspiration can cause permanent damage to the bronchileading to bronchiectasis. Patients with undiagnosedepiphrenic diverticula may also be at increased risk forcancer3, which occurs at a frequency of 0.3- 3% for pa-tients with this condition5.

Conclusion

Esophageal diverticulum can induce respira-tory symptoms due to regurgitation of food and subse-quent aspiration. This may lead to aspiration pneumo-nia, lung abscess and pleural complications. Apart fromthis, shadow of diverticulum filled with food and fluidmay mimic lung abscess. A clinical suspicion and inves-tigations such as barium contrast X-ray and upper GIendoscopy can demonstrate the diverticulum. The re-ported case presented to us clinically and radiologicallyas lung abscess and on further work up proved to beesophageal diverticulum.

References

1. Matthews BD, Nelms CD, Lohr CE, Harold KL,Kercher KW, Heniford BT. Minimally invasivemanagement of epiphrenic esophageal diverticula.Am Surg. 2003; 69:465-70.

2. Fasano NC, Levine MS, Rubesin SE, Redfern RO,Laufer I. Epiphrenic diverticulum: clinical andradiographic findings in 27 patients. Dysphagia. 2003;18:9-15.

3. Benacci JC, Deschamps C, Trastek VF, Allen MS,Daly RC, Pairolero PC. Epiphrenic diverticulum:results of surgical treatment. Ann Thorac Surg.1993;55:1109-113

4. Ravindran Chetambath, Jabeed Parengal,Mohammed Aslam, Sanjeev Shivashankaran.Esophagus Associated Lung Diseases- ARetrospective Study. JMSCR 2017; 5(3): 18289-96

5. Honda H, Kume K, Tashiro M, Sugihara Y, YamasakiT, Narita R, et al. Early stage esophageal carcinomain an epiphrenic diverticulum. Gastrointest Endosc.2003; 57:980-82.


Case Report

Renal Transplant Recipient with Cough

Amrutha Mukundan*, Paulo V. A.**, Anand M.***, Suraj K. P.*****Junior Resident, **Assistant Professor, *** Associate Professor, ****ProfessorDepartment of Pulmonary MedicineGovernment Medical CollegeKozhikode

Case Report

46 year old male who was on regular followupfor end stage renal disease and hypertension underwentcadaveric renal transplantation. Five days after hissurgery, he developed dry cough, low grade fever andhoarseness. He was on triple drug immunosuppressionwhich includes high dose steroids, tacrolimus andmycophenolate moefetil during post renal transplantperiod.

Que1 : What is the commonest cause of fever inimmediate (<1 month) post renal transplant period?

Bacterial infectionsViral infectionsTuberculosisGraft rejection

ANS : Bacterial infections3

Abstract

Aspergillus can cause a wide spectrum of disorders ranging from hypersensitivity reactions toinvasive diseases. Invasive pulmonary aspergillosis is common in immunocompromised. Itsincidence is found to be increasing over the years because of increasing number of patients withimpaired immunity especially with advanced HIV, haematological malignancy and solid organtransplantation1. A variant of this invasive infection called tracheobronchial aspergillosis is alsoknown to affect the major airways which is commonly described in neutropenic patients and lungtransplant recepients. Amongst the several types, pseudomembraneous tracheobronchial as-pergillosis can cause life threatening complications. Only one case of pseudomembraneoustracheobronchitis has been described in post renal transplant recipient so far2. Here we have asimilar case who survived due to the timely diagnosis and treatment with antifungal agents.

Key Words : Post Transplant, Immunosuppressed, Aspergillosis, Antifungals


Bacterial infections constitute the commonestcause of fever. It can be due to anatomical distortionspost surgery, urinary catheters, high dose steroids,aspiration and prolonged pretransplant hospitalization.

He was given broad spectrum antibiotics for 10days but his cough worsened and fever persisted.

On examination, he was conscious andoriented. Vitals were stable. System examination waswithin normal limits.

Que 2: Why early and detailed evaluation of fever isimportant in transplant recipients?

Graft rejectionBroad differential diagnosisAtypical clinical and radiological featuresMay results in graft failureHigh mortality due to concurrent immunosupressionAll of the above

ANS : All of the above

His blood routine showed low hemoglobin, mildleucocytosis with neutrophil predominence, raised ESRand normal renal function test. An ENT evaluation wasdone for his hoarseness. Video laryngoscopy showedonly mucosal edema of vocal cords.

A chest Xray taken at the onset of symptoms(fig1) was normal, but a repeat chest Xray a week later(fig2) showed a cavity in left upper zone.

Amrutha Mukundan - Renal Transplant Recipient with Cough

Fig 1

Fig 2

Fig 3 : HRCT thorax showed bilateral cavitating lesionswith feeding vessels, and irregularities within the

tracheal lumen and the right bronchus

Flexible video bronchoscopy revealed exten-sive nodular lesions covered with slough in trachea andboth bronchial trees. BAL and biopsy were taken. Bac-terial and AFB cultures of BAL were sterile. Fungal cul-ture showed greenish colonies of aspergillus fumigatus.Bronchial biopsy showed inflamed mucosa with as-pergillus colonies.



Fig No. 4 : Culture media showinggreenish colonies of Aspergillus fumigatus

So a final diagnosis of pseudomembraneoustracheobronchial aspergillosis/ invasive pulmonary as-pergillosis in a case of post renal transplant was con-sidered.

Que 5 : Most common organ involved in invasive as-pergillosis is

LungTracheaBrainSkinParanasal sinuses

Ans : Lung4

Lung constitute > 80 % of cases and sinuses 5-10%. Less common sites are brain, kidney and skin.

Invasive pulmonary aspergillosis is usuallyseen in immunocompromised patients.

A variant called invasive bronchial aspergillo-sis involves the large airways. It is classified by Den-ning etal into 3 types.

Superficial: Mucosal inflammation with noulcers or pseudomembrane.Ulcerative : Usually seen in post lungtransplants at the anastomotic sites. It is themost aggressive and is prone for fatalcomplications.Pseudomembraneous : Significant necrosis ofbronchial epithelium and formation ofpseudomembranes.

Que 6 : Which among the following describes the earli-est radiological feature seen in patient with invasivepulmonary aspergillosis?

Mediastinal nodesPleural effusionHalo signCrescent sign.

ANS : Halo sign

Halo sign is the ground glass opacity surround-ing a pulmonary nodule or mass and represents hem-



orrhage. It is typically seen in angioinvasive aspergillo-sis. It is a transient sign seen in early aspergillosis andis a good prognostic sign. It is neither sensitive nor spe-cific for IPA. It later evolves into air crescent sign oncethe immunity of the patient improves.5

Monitoring of the serum Aspergillusgalactomannan (GM) level has a role in the early diag-nosis of these cases6. Being a polysaccharide secretedby aspergillus, its concentration increases with increasein fungal burden. It is significant in neutropenic patients.

Voriconazole is the drug of choice for invasiveaspergillosis and far outweighs amphotericin B in viewof its safety profile7. Echinocandins can also be used asadjuvant to voriconazole but rarely used alone.

This patient was given liposomal amphotericinBfor 3 weeks followed by oral voriconazole for 4 months.A repeat scopy at 6weeks showed >80% resolution andat 10wks showed complete resolution.

Que 8 : What is the optimal duration of therapy in inva-sive pulmonary aspergillosis?

6 weeks• 6 months• 12 months• None of the above

ANS : None of the above

Optimum duration of treatment is not estab-lished. It should be individualized to the patient’s clini-cal and radiological response.

The prerequisites for discontinuation are clini-cal and radiological resolution, microbiological clear-ance and reversal of immunosuppression.8

Prognosis depends on immune status and un-derlying disease of the patient.

References

1. Mohan A, Guleria R, Mukhopadhyaya S,etalInvasive tracheobronchial aspergillosis in animmunocompetent person. The American Journalof Medical Sciences Feb 2005Volume 329, Issue 2,Pages 107–109

2. Abdullah Saymer, Seyhun Kursat, Huseyin Toz,Pseudomembraneous necrotising bronchialaspergillosis in a renal transplant recipient;Nephrology Dialysis Transplantation 1999 14:1784-1785

3. Kasper, Fauci, Hauser etal, Harrison’s Principles ofInternal Medicine 18th edition, McGraw-HillEducation, New York 2012 pg 1127

4. Kasper, Fauci, Hauser etal, Harrison’s Principles ofInternal Medicine 18th edition, McGraw-HillEducation, New York, 2012, page 1656

5. Gaeta M, BlandinoA, Scribano E, et al. Computedtomography halo sign in pulmonary nodules:frequency and diagnostic value. J Thoracic Imaging1999;14:109-113

6. Marcio Nucci, Simone A Nouer, Domenico Capponeetal, Early diagnosis of invasive pulmonaryaspergillosis in hematologic patients: an opportunityto improve the outcome.Haematologica November2013 98(11): 1657–1660.

7. Raoul Herbrecht, Denning DW, Patterson TF,et al.Voriconazole versus amphotericin B for primarytherapy of invasive aspergillosis. NEJM 2002; 347;408-415

8. Limper AH,Knox KS, Sarosi GA,et al. An officialAmerican Thoracic Society statement: treatmentof fungal infections in adult pulmonary and criticalcare patients. Am J Respir Crit Care Med 2011;183:96-128.






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