Interstitial Lung Disease

BY

DR. ANIMESH ARYA

CONSULTANT PULMONOLOGIST MAX HOSPITAL AND BALAJI ACTION

MEDICAL INSTITUTE NEW DELHI

58 YRS FEMALE OBESE H/O DOE FOR 2 YRS PROGRESSIVEADMITTED FOR INVESTIGATION AND BEING TREATED FOR HYPOXIC RESPIRATORY FAILURE

Lung window shows bilateral reticularity in the right and the left mid zones anterioly ( right>left ) with A haze suggestive of interestitial thickening with interestitial pnuemonitis.

Some alveolar opacities are also seen amidst the above suggestive of the alveolar exudates. The apical basal segments of both lower lobes also show reticularity with haze suggestive of interestitial thickening and interestitial pnuemonitis

The right lower zone shows an area of rounded cystic air spaces suggestive of areas of bronchiectasis. Some area of bronchiectasis are also seen in the above apical basal patches of pnuemonitis. A tiny emphysematous bulla is seen in the right upper zone peripharally.basal early honeycombing is seen.

Fob and lavage and TBLB Treatment with steroids Pulse therapy VS. regular dose OLB and the treat /not treat Supportive

THE LUMEN OF ALVEOLI SHOW DESQUAMMATED EPITHILIAL CELLS

THE WALL IS THICKENED EXPANDED AT CERTAIN FOCII BY COLLAGENOUS TISSUE WHILE AT PLACES THERE ARE LYMPHOID AGGREGATES.

CERTAIN ALVEOLAR SPACES SHOW PLASMA CELL, LYMPHOCYTES AND FEW HISTOCYTE COLLECTIONS.

THE TERMINAL BRONCHIOLES ARE THICKENED, WITH COLLAGENOUS WALL AROUND IT.

SECTION B FROM THE MIDDLE LOBE SHOW CERTAIN NORMAL LOOKING ALVEOLI WHILE AT PLACES THE INTERESTITIUM IS EXPANDED LYMPHOMONONUCLEAR CELLS

FEATURES OF DIP IN THE MIDDLE LOBE AND FEATURES OF DIP AND UIP IN THE LOWER

LOBE

A CRP diagnosis of UIP made with acute exacerbation

Treated with steroids 30 mg /day and azathioprine with oxygen and diuretics and LTOT

Uneventful course initially with worsening to resting hypoxia without oxygen and gr. IV dyspnoea

INCRESED ABDOMINAL FULLNESS, ACIDITY, FREQUENCY OF STOOLS AND SHORTNESS OF BREATH ON WALKING UPTO ABOUT A KM. GR I – II DYSPNOEA in may2006

NO H/O WHEEZING. CHEST PAIN H/OCOUGH, EXPECTORATION,SLIGHTLY YELLOWISH TO HGIC

NO PEDAL OEDEMA, NO CHEST PAIN , PND,ORTHOPNOEA NO SIGNIFICANT PAST HISTORY PATIENT IS EX SMOKER – 30 PACK-YEARS TILL ABOUT 6 MONTHS BACK ALCOHOLIC –LEFT SINCE 3 MONTHS

AS PER RECORD AVAILABLE WITH US GPE/ NAD, S/E NAD

PREVIOUS HISTORY- TMT IN MAY 2005- NAD REFERRED TO CHEST SPECIALST

HB-16.2 TLC-6800, P71,L22,M1,E6, ESR =10MM URINE –NAD BLOOD SUGAR 131 CXR

FVC=3.52/3.58 (98%) FEV1=2.83/2.88 (98%) FET==6.81 Seconds PEFR=8.08/7.02 lps (115%) FEF 25-75 =2.89/3.14 (92%)

Non contrast HRCT scan was done Multiple patchy foci of consolidation showing a predominantly

peripheral distribution seen at the lung bases bilaterally. The largest lesion is seen in the right lower lobe abutting the pleura with an adjacent area of ground glass density. Other lesions are significantly smaller and are seen in both the lower lobes as well as lingula and the middle lobe

B/L peripherally distributed patchy consolidation/infiltrates are also seen

No lymphadenopathy Radiological D/D – nonspecific shadows, ?infective, ?BOOP, ?EP,?OP

WAIT AND WATCH AS PT. IS MILDLY SYMPTOMATIC?

INVESTIGATE? WHICH INVESTIGATIONS?

CONSULTED ANOTHER CHEST PHYSICIAN AND TREATED WITH AB. FOR A WEEK AND REVIEW AGAIN AND INVESIGATE FOR AFB SMEAR AND CULTURE FOR THREE DAYS, AND SPUTUM CULTURE

SP CULTURE NEGATIVE, SP AFB NEG FOR 3 DAYS HIS COUGH WORSENED AND THE SPUTUM WAS MORE

BLOOD TINGED HE THEN REPORTED TO US FOR FOB AND

BIOPSY WITH INTENT TO RULE OUT MALIGNANCY (SUGGESTED BY SOMEBODY)

MX TEST, ACE LEVEL ,HIV, TEMP RECORD AND BRONCHOSCOPY

FOB PERFORMED MILD PURULENT SECRETIONS COMING OUT OF

APICAL AND POSTERIOR BASAL SEGMENTS OF RLL, APICAL SEGMENT WAS NAROWED

SENT FOR CYTOLOGY, AFB CULTURES AND TBLB TAKEN FROM THE RLL APICAL SEGMENT

CYTOLOGY, INCONCLUSIVE AFB SMEAR -NEG BIOPSY- A PIECE OF BRONCHIAL MUCOSA, IT

HAS PSEUDOSTRATIFIED LINING DISCONTINUOUS AT PLACES THERE IS SUBMUCOUSAL LYMPHONUCLEAR INFILTRATE, FEW FOAMY CELLS.AND A FEW ILL DEFINED GRANULOMAS

IMP: EPITHELOID GRANULOMATOUS INFLAMMATION BRONCHIAL TISSUE - ?CAUSE

TUBERCULOSIS ALVEOLAR SARCOID OP PUMONARY VASULITIS ???? IIP

PATIENT WAS TOLD OF THE REPORT AND HE DISCUSSED THIS WITH THREE DIFFERENT PULMONOLOGISTS AND THE OPINION WAS VARIED ABOUT

1. ATT, 2. ATT AND STEROIDS 3. STEROIDS 4 .WAIT AND WATCH FOR CULTURE

HOWEVER PATIENT WAS STARTED ON ANTITUBERCULAR TREATMENT WITH 4 DRUGS WITH THE CONSENT OF THE PT.

His symptoms worsened and the patient became dyspnoeic, with worsening hemoptysis and loss of weight

His CXR did not show much change , RATHER WORSENED

He was suggested to take steroids and discontinue ATT but he again went to at least three more pulmonologists and all agreed to same approach

In the mean time to get sputa for AFB for 3 more days, c-ANCA, p-ANCA, ACE levels, complement levels, repeat urine exam and ENT exam

All investigations came out to be normal. Meanwhile pt’s dyspnoea worsened.

He was suggested repeat CT scan thorax and PFT

FVC: 2.04 vs 3.52 FEV1: 1.61 vs

2.83

CT Report (18-8-05): There is marked volume loss of both lower lobes which show areas of collapse consolidatoion in both the lung bases. Evidence of thickened interstitium with ground glass density are also seen. Similar changes are evident in few areas of middle lobe and lingula. A few scattered infiltrates are seen in the upper lobes but these portions of lung are mostly spared. There is no lymph nodes and or pleural effusion

There is marked progression of lesions as compared to the previous scan of 30-6-05.

No definitive radiological opinion was volunteered.

Patient was advised to undergo OLB at this stage for a conclusive diagnosis

Pt refused procedure and continued on steroids Went to AIIMS and subjected to FOB and TBLB

again ON 26/08/05 Biopsy result-shows respiratory epithelium,

subepithelium showing mild thickening of the interalveolar septae and sparse chronic inflammatory infiltrate. The possibility of inflammatory lung disease can not be ruled out in the small biopsy.

REPEAT FOB WITH BAL OPEN LUNG BIOPSY AND MICROBIOLOGICAL

SAMPLING CHANGE MX WITH ADDITION OF

IMMUNOSUPPRESSIVES

D/D ?? Continued on steroids and we reemphasised the

need for a firm diagnosis by larger biopsy by way of OLB

Patient undergoes open lung biosy on 14/09/05 at another institute after consulting five pulmonologists in Delhi and Bombay

Reported as widening of the interstitial tissue which is infiltrated by the inflammatory cells. Some of the alveolar lumina contain macrophages and chronic inflammatory cells consisting of lymphocytes - Intersit ial Pneumonitis of Lung

Review of OLB in AIIMS - Compatible with organising pneumonia. No granuloma seen

Review at another institution - UIP

INTERSTITIAL PNEUMONIA VARIETY?

UIP NSIP-SUBTYPE=

CELLULARFIBROTICMIXED -OP-FIBROTIC NSIP

After OLB patient was also given azathioprine besides steroids

Meanwhile AFB culture on lung biopsy and bronchial aspirate came out to be negative

WAS THE CLINICAL DIAGNOSIS CORRECT? DID WE MISS AT ANY INVESTIGATION? WAS APPROACH CORRECT? FOB AND BIOPSY WERE INADEQUATE AND

CONFUSING OLB DID IT GIVE US THE FINAL ANSWER? VARIANCE OF VARIOUS PATHOLOGISTS

IMPRESSION

NSIP OP-NSIP ?? USUAL INTERSTITIAL PNEUMONITIS ?? UIP WITH COP ?? COP ???? OTHER

DIFFICULT TO ARRIVE AT A DIAGNOSIS BETTER CLINICAL , RADIOLOGIC AND SURICAL

CORELATION IS MANDATORY OPEN LUNG BIOPSY IS THE NEED OF THE HOUR ,

CAN BE EASILY PERFORMED BUT PERHAPS THE APPROPRIATE INTERPRETATION OF THE FINDINGS IS STILL ELUSIVE AND MAY NOT BE THE GOLD STANDARD

48 MALE, NON SMOKER DOE -2MONTHS REVIEWD BY PHYSICIANS NO DIAGNOSIS CXR – BASAL LINEAR SHADOWS

LOSS OF LUNG VOLUMEEXAM –BIBASILAR FINE CRACKLES

ADVISED CT SCAN CHEST AND PFT

PATCHY GROUND GLASS LOSS OF LUNG VOLUMES NO THICKENING OF INTERSTITIUM NO HONEY COMB INTERSTITIAL PNEUMONITIS-NSIP PFT – RESTRICTIVE DISORDER

FOB AND LAVAGE – INCONCLUSIVE TREATMENT AS CELLULAR NSIP

PULSE 1 GM OF METHYLPREDNISOLONE X 3 DAYS AND THEN

START ON 10 MG WYSOLONE AND AZATHIOPRINE 50 MG/DAY

DOE –HIGH INDEX OF SUSPICION MUST R/OOTHER DISEASES A HIGH QUALITY CT-WITH A CR CONF AGOOD PFT WITH DLCO MANDATORY TIME PLANE HIGH PROBABLITY OF IIP ALGORITMIC APPROACH IF DIA CERTAIN NO NEED FOR OLB , FOB COULD BE DONE TO

R/O INFECTIONS BAL ROLE IN INDIA IF OLB ATTEMPED SELECT THE SITE NO. SIZE AND GOOD

PATHOLOGIST

TREATMENT AIM AT WHAT POTENTIAL CURE CELLULAR/GG-TREAT /PULSES FIBROTIC –NO GOOD ONLY SUPPORTIVE NEWER DRUGS-NAC /OTHERS