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11/7/2018
1
Interstitial Lung Disease:Clinical Approach to Diagnosis
Rupal J. Shah, MD MSDirector, Interstitial Lung Disease ClinicUniversity of California, San Francisco
Disclosures
• Nothing to Disclose
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Outline
• What is ILD?
• Diagnostic Approach
• Specific types of ILD
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What is the pulmonary interstitium?
• Anatomic space that is lined by epithelial and endothelial cells
• Contains collagen, elastin, reticulin, ECM
• Also in the connective tissue of the lung (interlobular septa, visceral pleura, peribronchovascularsheaths)
Not Just Interstitial
• Misnomer because many ILD’s affect the airways, parenchyma, blood vessels and pleura
• More accurately described as diffuse parenchymal lung disease
• Over 100 types of ILD’s
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Farrand, E et al The hospitalized patient with interstitial lung disease: A hospitalist primer J Hosp Med 2017
Epidemiology
• Very difficult to estimate
• Prevalence:
– IPF: 10‐40/100,000 adult per year
– All ILD: 60‐80/100,000 adults per year
• COPD: 5% of the population (16 million)
Raghu, et al Incidence and prevalence of IPF AJRCCM 2006Coultas DB The epidemiology of interstitial lung disease AJRCCM 1994
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Epidemiology
Lederer DJ, et al Idiopathic pulmonary fibrosis NEJM 2018
Diagnosis
• Challenge: Presentation is usually nonspecific
• Average time from symptom onset to diagnosis: 1‐2 years
• Early recognition is important!
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Outline
• What is ILD?
• Diagnostic Approach
• Specific types of ILD
Clinical Evaluation: History
12
Elements Examples Demographics
Age: IPF>50 years, 20-40: CTD, sarcoid, LAM, LCH, familial Female: LAM, CTD-ILD, Men: RA-ILD, occupational
Onset of Symptoms Timing can guide the differential (acute: COP, CTD-ILD, chronic: IPF, sarcoid)
Smoking History LCH, DIP, RB-ILD, IPF
Family History Premature graying, history of ILD
Review of Systems Arthralgias, Raynaud’s, Myositis, Dysphagia/Reflux
Drugs Occupational/Environmental Exposures
Pneumotox.com Don’t forget OTC and illicit Birds/Mold Asbestos, Silica Next Slide
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Clinical Evaluation: Physical Exam
14Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
Elements Examples Lung
Nonspecific, typically crackles or rales
Cardiac Signs of pulmonary HTN Edema, augmented P2, RV heave
Extremities Clubbing, joint deformities, weakness
Skin Alopecia, angiofibromas, Gottron’s papules, mechanic’s hands, sclerodactyly (next slide)
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Clinical Evaluation: Physical Exam
15Folliculofibroma: BHD Angiofibroma: LAM
Diagnostic Tests
• Imaging (to be discussed by Dr. Elicker)
• Pulmonary Function Tests
• Laboratory
• Bronchoscopy
• Surgical lung biopsy
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PFT Interpretation
Order full PFT’s
Pulmonary Function Test
Spirometry Predicted Observed %PredFVC 3.72 2.24 60%FEV1 3.06 1.78 58%FEV1/FVC 82 79 96%
PlethysmographyTLC 5.26 3.38 64%
DiffusionDiffusing Capacity 29.01 8.01 28%
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Clinical Evaluation: Laboratory Analysis
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Elements Comment
CBC with differential Macrocytosis (telomeropathy)Eosinophilia (CEP)
Autoimmune serologies Next slide
HP precipitans Poor sensitivity and specificity, limited range of antigens tested
Genetic testing Selected cases (e.g. BHD), emerging for FPF
Telomere length measurement Emerging
VEGF‐D Lymphangioleiomyomatosis
Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM 2013
Autoimmune Serologies
• Initial Evaluation
– ANA
– RF/CCP
– ANCA
– Myositis Panel
– SSa/SSb
Alsumrain, M Utility of autoimmune serology testing in the assessment of uncharacterized ILD: a large retrospective cohort review Respir Res 2017
*yield is lower with increasing age
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Bronchoscopy
Meyer KC, Raghu G. Bronchoalveolar lavage for the evaluation of interstitial lung disease: is it clinically useful? Eur Respir J. 2011;38:761-769.
Surgical Lung Biopsy• Mortality
– 1.7% (elective)– 16% (non‐elective)
22Hutchinson,JP et al In-Hospital Mortality after SLB for ILD in the US 2000-2011 AJRCCM 2015
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History, Physical Exam,CT scan, labs
Diagnosis!IPFHP (some)LCH, AP, LAMCTD‐ILDSome occupational lung diseases
Is bronchoscopy safe and indicated?SarcoidMalignancyEosinophilic pneumoniaCOP
NoSurgical Lung Biopsy
Bronchoscopy Diagnostic
Confident diagnosis
Diagnosis of highest probability (MDC)
Procedures are unsafe
Non diagnostic
Non diagnostic
Adapted from Wells, AU ILD guideline: the BTS with Thoracic society of Australia and New Zealand and Irish Thoracic Society Thorax 2008
Multi Disciplinary Conference
Flaherty KR, King TE, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170:904-910.
• Agreement is best when there is a consensus discussion between clinicians, radiology, and pathology
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Outline
• What is ILD?
• Diagnostic Approach
• Specific types of ILD
• Available therapies
• Cases
Idiopathic Pulmonary Fibrosis• IPF is a specific form of chronic,
progressive fibrotic interstitial lung disease that occurs in older adults and is characterized by radiographic or pathologic usual interstitial pneumoniawithout a secondary cause
• UIP pattern: peripheral basilar reticulation, traction bronchiectasis and honeycombing without other features (i.e. ground glass, air trapping, etc)– CTD, asbestosis, chronic HP, XRT
11/7/2018 26Official ATS/ERS/JRS/ALAT Guidelines on Idiopathic Pulmonary Fibrosis. AJRCCM 2011;183:788
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IPF: Why is diagnosis important?
• Median survival ~4 years
• Acute exacerbation rate 5‐10% per year
27Ryerson CJ et al Predicting survival across interstitial lung disease: the ILD-GAP model Chest 2014
Hypersensitivity Pneumonitis• No consensus diagnostic criteria
– Classic imaging + good exposure
– Lung biopsy
• Described as acute, sub-acute, and chronic
• Over 200 exposures identified:– Typically related to mold or bird products
• Treatment– Remove exposure
– Prednisone +/- mycophenolate or azathioprine
28
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Connective Tissue Disease
29
CTD Diagnosis
Systemic Sclerosis
Esophageal Dilation
Rheumatoid Arthritis Consider drug induced lung injury for new/worsening ILD
Dermatomyositis/Polymyositis Myositis may be subtle ILD can come first ANA can be negative
Sjogren’s Syndrome Cysts on HRCT
Lupus ILD is unsual
Adapted from Vij, R Diagnosis and Treatment of CTD-ILD Chest 2013
Interstitial Lung Abnormalities• Increased lung density on CT scans in patients with no history of ILD
• 2‐10% of the population• Association with reduction in PFT, exercise capacity, development of ILD, and mortality
• Refer to pulmonary
Putman, RK et al Association between ILA and All‐cause mortality JAMA 2016
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Conclusions
• Multiple types of ILD
• Consider in older patient with cough or subacute shortness of breath
• Evaluation starts with high resolution CT, may need surgical lung biopsy
• Important to distinguish IPF from other types of ILD as treatment differs
Refer to us!
• UCSF ILD Program
• https://www.ucsfhealth.org/clinic/interstitial_lung_disease_program/#
• 415.353.2577
Environmental Exposure Assessment Form
Interstitial lung disease (ILD) can be a progressive scarring disease of the lung. Some types of ILD can be related to environmental exposures. UCSF’s Interstitial Lung Disease Program developed this assessment form to help gauge your potential exposures. This checklist does not include every possible exposure and having an exposure does not mean that it is the cause of your lung disease. This form is best used in consultation with an expert in interstitial lung disease.
General
Approximate year your house/residence was built: _________________
Address: ______________________________________________________________
Is the location: 1. Urban 2. Suburban 3. Rural
Race: 1. Caucasian 2. Hispanic 3. African American 4. Asian 5. Other (specify)
Are you an immigrant?
If yes, from which country/region? 1. Mexico 2. Asia 3. Canada 4. Other (specify) Yes No
Does your residence or office smell musty? Yes No
Has there been a history of flooding? Yes No
Is there water damage to the walls or ceilings
(Look behind furniture that is up against the walls in your rooms)? Yes No
Do you have carpeting?
If yes, When was it installed? __________
Is the carpeting steam-cleaned regularly?
Was the carpeting installed over cement?
Yes No
Yes No
Yes No
Do you have a hot tub or sauna? How often do you use it? ___________ Yes No
Are there any birds (pets or nesting) on the property?
If yes, which species? 1. Finch 2. Cockatiel 3. Parakeet 4. Other (specify)
How long was the exposure? _______ days/weeks/months/years
Was the bird(s) kept indoors?
Yes No
Yes No
Do you have any decorations, clothing or furniture with bird feathers (e.g. down feathers)? Yes No
Do you have a lot of plants in your home or office? Yes No
Do you have a fish tank(s)? Yes No
Do the window sills in your home of office show any signs of mold? Yes No
Do you use a swamp cooler? Yes No
Do you use humidifiers, dehumidifiers or vaporizers? Yes No
Do you use ozone cleaners, HEPA filters or other air filters? Yes No
Are there any water features, such as a fountain, in your home? Yes No
Do you have a water collection system, cistern or water storage unit? Yes No
Heating your residence
Do you have central or forced-air heating
If yes, is there mold on filters? Yes No
Do you use a wood-burning stove at least once a week? Yes No
Kitchen
Do you have any appliances or sinks that leak water or have a water pan to change? Yes No
Does your dishwasher leak/overflow or smell musty? Yes No
To refer a patient: Interstitial Lung Disease Program • Phone 415-353-2577 • Fax 415-353-8944 Lung Transplant Program • Phone 415-353-4145 • Fax 415-353-4166Referral forms can be found at www.ucsfhealth.org/referral
To refer a patient: Interstitial Lung Disease Program • Phone 415-353-2577 • Fax 415-353-8944 Lung Transplant Program • Phone 415-353-4145 • Fax 415-353-4166Referral forms can be found at www.ucsfhealth.org/referral
Bathroom
Are there signs of mold or mildew on walls, ceilings, tiles or curtains? Yes No
Is there a working exhaust fan? Yes No
Bedrooms
Do you have any clothes, jackets, pillows or comforters that contain down (fine bird feathers)? Yes No
Do you use a Sleep Number® (or similar) bed or water bed? Yes No
Closets
Are any leather clothes or shoes stored in your closets covered with a white or black film? Yes No
Are the walls of the closets discolored or are they covered with a black or white film? Yes No
Other rooms in your home
Does your clothes washing machine smell musty (if you have one in your residence)? Yes No
Is there a hobby room or workshop in or attached to your home, including a garage used in this way? Yes No
Are there any rooms located below ground or built on the side of a hill? Yes No
Basement
Is there any mold or mildew on walls or boxes? Yes No
Are there times during the year when water gets into the basement? Yes No
Do you spend time in the basement? Yes No
Is there carpeting in the basement?
If yes, was it installed over cement? Yes No
Yes No
Outside your home
Is there a compost pile in or near your home? Yes No
Is there any evidence of birds roosting on the roof, rafters, sills or decks? Yes No
Outdoor activities
Do you work with potting soils, moist soil or compost on a regular basis? Yes No
Do you hunt in duck blinds or have exposure to a lot of feathers? Yes No
Have you been exposed to moldy hay or grain? Yes No
Have you been involved in the packaging industry? Yes No
Have you been a farm worker?
If yes, for how many years? Yes No
Please list the products cultivated (e.g. almonds, grapes, etc.).
Have you been exposed to pesticides and/or other chemicals? Yes No
Did you ever smoke?
If yes: Packs/day: _________ Number of years: _________
Do you currently smoke?
If no, when did you quit? ____________________
Have you ever lived with a smoker?
If yes, for how long? ____________________________
Yes No
Yes No
Yes No
Name_________________________________ Date Completed_______________ revised 07.18