Interstitial Lung Disease: Clinical Approach to Diagnosis

Transcription

1 Interstitial Lung Disease: Clinical Approach to Diagnosis Rupal J. Shah, MD MS Director, Interstitial Lung Disease Clinic University of California, San Francisco Nothing to Disclose Disclosures 1

2 What is ILD? Diagnostic Approach Specific types of ILD Outline 2

3 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, peribronchovascular sheaths) 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 3

4 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 2006 Coultas DB The epidemiology of interstitial lung disease AJRCCM

5 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! 5

6 What is ILD? Diagnostic Approach Specific types of ILD Outline Clinical Evaluation: History Elements Demographics Examples Age: IPF>50 years, 20-40: CTD, sarcoid, LAM, LCH, familial Female: LAM, CTD-ILD, Men: RA-ILD, occupational Onset of Symptoms Smoking History Family History Review of Systems Drugs Timing can guide the differential (acute: COP, CTD-ILD, chronic: IPF, sarcoid) LCH, DIP, RB-ILD, IPF Premature graying, history of ILD Arthralgias, Raynaud s, Myositis, Dysphagia/Reflux Pneumotox.com Don t forget OTC and illicit Occupational/Environmental Exposures Birds/Mold Asbestos, Silica Next Slide 12 6

7 Clinical Evaluation: Physical Exam Elements Lung Cardiac Extremities Skin Examples Nonspecific, typically crackles or rales Signs of pulmonary HTN Edema, augmented P2, RV heave Clubbing, joint deformities, weakness Alopecia, angiofibromas, Gottron s papules, mechanic s hands, sclerodactyly (next slide) Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM

8 Clinical Evaluation: Physical Exam Folliculofibroma: BHD Angiofibroma: LAM 15 Diagnostic Tests Imaging (to be discussed by Dr. Elicker) Pulmonary Function Tests Laboratory Bronchoscopy Surgical lung biopsy 8

9 PFT Interpretation Order full PFT s Pulmonary Function Test Spirometry Predicted Observed %Pred FVC % FEV % FEV1/FVC % Plethysmography TLC % Diffusion Diffusing Capacity % 9

10 Clinical Evaluation: Laboratory Analysis Elements CBC with differential Autoimmune serologies HP precipitans Genetic testing Telomere length measurement VEGF D Comment Macrocytosis (telomeropathy) Eosinophilia (CEP) Next slide Poor sensitivity and specificity, limited range of antigens tested Selected cases (e.g. BHD), emerging for FPF Emerging Lymphangioleiomyomatosis Travis, WD et al An official ATS/ERS statement: Update of the international multidisciplinary classification of IIP AJRCCM Autoimmune Serologies Initial Evaluation ANA RF/CCP ANCA Myositis Panel SSa/SSb *yield is lower with increasing age Alsumrain, M Utility of autoimmune serology testing in the assessment of uncharacterized ILD: a large retrospective cohort review Respir Res

11 Bronchoscopy Meyer KC, Raghu G. Bronchoalveolar lavage for the evaluation of interstitial lung disease: is it clinically useful? Eur Respir J. 2011;38: Mortality 1.7% (elective) 16% (non elective) Surgical Lung Biopsy Hutchinson,JP et al In-Hospital Mortality after SLB for ILD in the US AJRCCM

12 Diagnosis! IPF HP (some) LCH, AP, LAM CTD ILD Some occupational lung diseases History, Physical Exam, CT scan, labs Is bronchoscopy safe and indicated? Sarcoid Malignancy Eosinophilic pneumonia COP Procedures are unsafe No Non diagnostic Diagnosis of highest probability (MDC) Non diagnostic Surgical Lung Biopsy Bronchoscopy Diagnostic Adapted from Wells, AU ILD guideline: the BTS with Thoracic society of Australia and New Zealand and Irish Thoracic Society Thorax 2008 Confident diagnosis Multi Disciplinary Conference Agreement is best when there is a consensus discussion between clinicians, radiology, and pathology 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:

13 What is ILD? Diagnostic Approach Specific types of ILD Available therapies Cases Outline 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 pneumonia without 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 26 Official ATS/ERS/JRS/ALAT Guidelines on Idiopathic Pulmonary Fibrosis. AJRCCM 2011;183:788 13

14 IPF: Why is diagnosis important? Median survival ~4 years Acute exacerbation rate 5 10% per year Ryerson CJ et al Predicting survival across interstitial lung disease: the ILD-GAP model Chest 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 14

15 Connective Tissue Disease CTD Systemic Sclerosis Rheumatoid Arthritis Dermatomyositis/Polymyositis Sjogren s Syndrome Lupus Diagnosis Esophageal Dilation Consider drug induced lung injury for new/worsening ILD Myositis may be subtle ILD can come first ANA can be negative Cysts on HRCT ILD is unsual Adapted from Vij, R Diagnosis and Treatment of CTD-ILD Chest 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

16 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 terstitial_lung_disease_program/#

17 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) Does your residence or office smell musty? Has there been a history of flooding? Is there water damage to the walls or ceilings (Look behind furniture that is up against the walls in your rooms)? Do you have carpeting? If yes, When was it installed? Is the carpeting steam-cleaned regularly? Was the carpeting installed over cement? Do you have a hot tub or sauna? How often do you use it? 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? Do you have any decorations, clothing or furniture with bird feathers (e.g. down feathers)? Do you have a lot of plants in your home or office? Do you have a fish tank(s)? Do the window sills in your home of office show any signs of mold? Do you use a swamp cooler? Do you use humidifiers, dehumidifiers or vaporizers? Do you use ozone cleaners, HEPA filters or other air filters? Are there any water features, such as a fountain, in your home? Do you have a water collection system, cistern or water storage unit? Heating your residence Do you have central or forced-air heating If yes, is there mold on filters? Do you use a wood-burning stove at least once a week? Kitchen Do you have any appliances or sinks that leak water or have a water pan to change? Does your dishwasher leak/overflow or smell musty? To refer a patient: Interstitial Lung Disease Program Phone Fax Lung Transplant Program Phone Fax Referral forms can be found at

18 Bathroom Are there signs of mold or mildew on walls, ceilings, tiles or curtains? Is there a working exhaust fan? Bedrooms Do you have any clothes, jackets, pillows or comforters that contain down (fine bird feathers)? Do you use a Sleep Number (or similar) bed or water bed? Closets Are any leather clothes or shoes stored in your closets covered with a white or black film? Are the walls of the closets discolored or are they covered with a black or white film? Other rooms in your home Does your clothes washing machine smell musty (if you have one in your residence)? Is there a hobby room or workshop in or attached to your home, including a garage used in this way? Are there any rooms located below ground or built on the side of a hill? Basement Is there any mold or mildew on walls or boxes? Are there times during the year when water gets into the basement? Do you spend time in the basement? Is there carpeting in the basement? If yes, was it installed over cement? Outside your home Is there a compost pile in or near your home? Is there any evidence of birds roosting on the roof, rafters, sills or decks? Outdoor activities Do you work with potting soils, moist soil or compost on a regular basis? Do you hunt in duck blinds or have exposure to a lot of feathers? Have you been exposed to moldy hay or grain? Have you been involved in the packaging industry? Have you been a farm worker? If yes, for how many years? Please list the products cultivated (e.g. almonds, grapes, etc.). Have you been exposed to pesticides and/or other chemicals? 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? Name Date Completed revised To refer a patient: Interstitial Lung Disease Program Phone Fax Lung Transplant Program Phone Fax Referral forms can be found at