Interstitial Pulmonary Fibrosis A Deadly Disease. Said Chaaban, MD - PowerPoint PPT Presentation

Interstitial Pulmonary Fibrosis A Deadly Disease. Said Chaaban, MD Assistant Professor of Medicine Pulmonary and Critical Care Medicine University of Kentucky August, 2018

Disclosure • This presentation has no: • Conflicts of interest • Off-label promotion of medications • PI: PF-ILD

“. . ..some kind of fibrosis. . .. . ..so I just kept going to the clinic and they did lung function. . ..which. . ..at the time. . ..were quite low- . . ..then he decided to refer me to Dr. X’s clinic and then we found out that I was too bad for the trial [Bridget]” 1. Duck A, Spencer LG, Bailey S, Leonard C, Ormes J, Caress AL. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis. J Adv Nurs. 2015;71(5):1055-65.

What is IPF? • Specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause occurring in adults • Exclusion of other forms of interstitial pneumonia 1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304. 2. Tcherakian C, Cottin V, Brillet PY, et al. Progression of idiopathic pulmonary fibrosis: lessons from asymmetrical disease. Thorax. 2011;66(3):226-31.

Diffuse Parenchymal Lung Disease DPLD of known cause Granulomatous Idiopathic Other forms of DPLD e.g. drugs or association DPLD e.g. Interstitial e.g. LAM, HX etc. e.g. collagen vascular disease sarcoidosis Pneumonias Idiopathic Pulmonary IIP other than Idiopathic Fibrosis pulmonary Fibrosis Respiratory bronchiolitis Desquamative Interstitial Interstitial lung disease Pneumonia Acute Interstitial Cryptogenic Pneumonia Organizing pneumonia Nonspecific interstitial Lymphocytic interstitial Pneumonia (provisional) Pneumonia 1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.

Is it Common? 1. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014;2(7):566-72.

1. Raghu G, Chen SY, Yeh WS, et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014;2(7):566-72.

Who is at risk? • Men • GERD • Age > 65 (rare <50 years) • Familial (5-10%) • Cigarette smoking • Occupational exposures: • Farming • Livestock • Raising birds • Wood (brass, lead, and steel) • Metal dust (pine) • Hair dressing • Stone cutting/polishing • Vegetable dust/Animal dust • Viral infections • Human herpes viruses (CMV, EBV, and HHV-8) • Hepatitis C 1. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017 2. Taskar VS, Coultas DB. Is idiopathic pulmonary fibrosis an environmental disease?. Proc Am Thorac Soc. 2006;3(4):293-8. 3. Maher TM, Wells AU, Laurent GJ. Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms?. Eur Respir J. 2007;30(5):835-9. 4. Mora AL, Rojas M, Pardo A, Selman M. Emerging therapies for idiopathic pulmonary fibrosis, a progressive age-related disease. Nat Rev Drug Discov. 2017;16(11):755-772 5. Ghebre YT, Raghu G. Idiopathic Pulmonary Fibrosis: Novel Concepts of Proton Pump Inhibitors as Antifibrotic Drugs. Am J Respir Crit Care Med. 2016;193(12):1345-52.

1. Steele MP, Schwartz DA. Molecular mechanisms in progressive idiopathic pulmonary fibrosis. Annu Rev Med. 2013;64:265-76.

1. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017;

“. . .it started with a cough’ ‘. . . ‘I was getting a bit breathless going upstairs’ being typical. From there on, the journey to diagnosis was variable, usually causing anxiety and frustration. Participants described a lengthy delay from being treated in primary care initially for asthma, COPD or recurrent chest infections before being referred to a local chest physician” 1. Duck A, Spencer LG, Bailey S, Leonard C, Ormes J, Caress AL. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis. J Adv Nurs. 2015;71(5):1055-65.

Increase Awareness… • Education in primary care: • Avoid diagnostic delays • Raise awareness of IPF • Kaplan-Meier plot of survival probability: • Time of the onset of symptoms • Median survival was 80.8 mo (65.5–89.3) • Time from initial visit • Median survival was 35.2 mo (23.2–48.5) 1. Duck A, Spencer LG, Bailey S, Leonard C, Ormes J, Caress AL. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis. J Adv Nurs. 2015;71(5):1055-65. 2. Kim DS, Collard HR, King TE. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3(4):285-92.

Clinical Presentation • Dyspnea upon exertion (gradual) • Non-productive (dry) cough • Reduced exercise tolerance • Increased anxiety • Fatigue • Depression Less common • Chest pain 1. Loveman E, Copley VR, Colquitt J, et al. The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation. Health Technol Assess. 2015;19(20):i-xxiv, 1-336. 2. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.

Physical examination • Inspiratory crackles (lung bases posteriorly) • Finger Clubbing (50%) • Signs of connective tissue disease • Peripheral edema (RHF) • Joint swelling or tight skin  collagen vascular disease 1. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017; 2. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.

Noninvasive Testing • FEV1/FVC (Normal or increased) • FVC (Reduced) • TLC (Reduced) • DLCO (Reduced) 1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.

1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277-304.

1. Dr. Google

Workup… • Bronchoscopy with BAL (weak recommendation) • Transbronchial biopsy (weak recommendation) • Serological evaluation, important in majority of patients (weak recommendation) • Perform rheumatoid factor, anti-cycliccitrullinated peptide, and anti-nuclear antibody titer and pattern 1. Raghu G, Rochwerg B, Zhang Y, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192(2):e3-19.

Diagnostic Criteria • Exclusion of other known causes of Specific combinations UIP pattern on ILD: of HRCT and surgical HRCT • Domestic and occupational lung biopsy environmental exposures pattern • Connective tissue disease • Drug toxicity 1. Raghu G, Rochwerg B, Zhang Y, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192(2):e3-19.

Suspected IPF Identifiable cause for Not IPF ILD No HRCT 1. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017;

1. Flaherty KR, Thwaite EL, Kazerooni EA, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax. 2003;58(2):143-8.

1. Webb WR, Muller NL, Naidich DP. High-Resolution CT of the Lung. Lww; 2014.

1. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.