Disclosures Nothing to Disclose Interstitial Lung Disease: - PowerPoint PPT Presentation

6/22/2018 Disclosures • Nothing to Disclose Interstitial Lung Disease: Decoding the Alphabet Soup Rupal J. Shah, MD MS Director, Interstitial Lung Disease Clinic University of California, San Francisco Outline • What is ILD? • Diagnostic Approach • Specific types of ILD • Available therapies • Cases 1

6/22/2018 Not Just Interstitial What is the pulmonary interstitium? • Misnomer because many ILD’s affect the • Anatomic space that is lined by airways, parenchyma, blood vessels and epithelial and endothelial cells • Contains collagen, elastin, reticulin, pleura ECM • More accurately described as diffuse • Also in the connective tissue of the lung (interlobular septa, visceral parenchymal lung disease pleura, peribronchovascular • Over 100 types of ILD’s sheaths) Epidemiology Farrand, E et al The hospitalized patient with interstitial lung disease: A hospitalist primer J Hosp Med 2017 Lederer DJ, et al Idiopathic pulmonary fibrosis NEJM 2018 2

6/22/2018 Diagnosis Outline • Challenge: Presentation is usually nonspecific • What is ILD? • Average time from symptom onset to • Diagnostic Approach diagnosis: 1-2 years • Specific types of ILD • Early recognition is important! • Available therapies • Cases 67 yo M with progressive dyspnea and Clinical Evaluation: History cough. Treated with abx for bronchitis. 85% 40 pack year smoker, quit 6 years ago. Elements Examples On exam, late inspiratory crackles, Demographics Age, IPF > 50 +clubbing. CXR shows increased basilar reticulation. Time course Acute, sub-acute, chronic What additional historical information is Extra-pulmonary symptoms of CTD Raynauds, rash, inflammatory arthritis, proximal muscle weakness, dry eyes/mouth most likely to assist in establishing a Smoking history DIP, RB-ILD, LCH, AEP diagnosis? 15% Medications/Radiation Nitrofurantoin, Amiodarone, methotrexate, chemotherapy, 0% 0% radiation 77 medications (pneumotox.com) A. Allergy History HP exposures (home, work, hobbies) Avian (birds, down), molds (water damage, swamp cooler), B. Family History mycobacteria ( indoor hot tub , metal working fluid) y y y y r r r r o o o o t t t t C. Occupational History s s s s Occupational exposures Asbestos, beryllium, metal dusts i i i i H H H H y l l y a e g l i n v D. Travel History r m e o a Family history of ILD Early graying, cryptogenic cirrhosis, bone marrow disorders a r l i l t T A F a p u c Travis, WD et al An official ATS/ERS statement: Update of the c O 12 international multidisciplinary classification of IIP AJRCCM 2013 3

6/22/2018 Clinical Evaluation: Physical Exam Clinical Evaluation: Physical Exam (images) Elements Examples Face and mucus membranes Scleritis, saliva pool & dentition, oral ulcers, leukoplakia, mouth opening, squared off telangectasias, fibrofolliculomas, malar rash Lungs Dry crackles (listen at bases or may miss early disease!) Inspiratory squeaks (bronchiolitis) Signs of pulmonary hypertension Cardiac Clubbing, mechanics hands, sclerodactyly, nail bed capillaries, Hands palmar telangectasias, Gottrons papules, synovitis and deformities, dystrophic nails Proximal muscle weakness Neuro Travis, WD et al An official ATS/ERS statement: Update of the 13 14 international multidisciplinary classification of IIP AJRCCM 2013 Diagnosis CXR • Imaging • Pulmonary Function Tests • Laboratory • Bronchoscopy • Surgical lung biopsy 4

6/22/2018 CT chest • High resolution CT scan with inspiratory and expiratory images Reticulation Nodular Pattern 5

6/22/2018 Bronchiectasis Air Trapping Cysts Ground Glass Consolidation 6

6/22/2018 HRCT guides differential PFT Interpretation Nodular Fibrotic Cystic • • • Perilymphatic IPF LAM Order • • • CTD-ILDs LCH Sarcoidosis full • • • LIP PFT’s cHP LIP • • • Amyloidosis Others BHD • Pneumoconioses • Lymphangitic Consolidation/GGO carcinomatosis • NSIP • Centrilobular • OP • HP, RB, FB, LCH • AEP/CEP • Infection 25 Pulmonary Function Test Flow Volume Loop Spirometry Predicted Observed %Pred FVC 3.72 2.24 60% FEV1 3.06 1.78 58% FEV1/FVC 82 79 96% Plethysmography TLC 5.26 3.38 64% Diffusion Diffusing Capacity 29.01 8.01 28% 7

6/22/2018 Clinical Evaluation: Laboratory Analysis 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 29 Respir. Med. 2016;113:80-92 30 international multidisciplinary classification of IIP AJRCCM 2013 Bronchoscopy Surgical Lung Biopsy • Mortality – 1.7% (elective) – 16% (non-elective) Hutchinson,JP et al In-Hospital Mortality after SLB for ILD in Meyer KC, Raghu G. Bronchoalveolar lavage for the evaluation of interstitial lung 32 disease: is it clinically useful? Eur Respir J. 2011;38:761-769. the US 2000-2011 AJRCCM 2015 8

6/22/2018 History, Physical Exam, Procedures Multi Disciplinary Conference CT scan, labs are unsafe Diagnosis of highest probability Diagnosis! Is bronchoscopy safe Non IPF diagnostic and indicated? HP (some) No Sarcoid Surgical Lung Biopsy LCH, AP, LAM Malignancy Some occupational lung Non Eosinophilic pneumonia diseases diagnostic COP Bronchoscopy Diagnostic • Agreement is best when there is a consensus discussion between clinicians, radiology, and pathology Confident diagnosis Adapted from Wells, AU ILD guideline: the BTS with Thoracic society of Australia and New Zealand and Irish Flaherty KR, King TE, Raghu G, et al . Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach Thoracic Society Thorax 2008 to diagnosis ? Am J Respir Crit Care Med. 2004;170:904-910. Outline Focus on fibrotic lung disease • What is ILD? • Diagnostic Approach Idiopathic Connective Hyper- • Specific types of ILD Pulmonary Tissue sensitivity Fibrosis Disease Pneumonitis Other • Available therapies • Cases Antifibrotics Immunomodulation Extrapulmonary dz / antigen avoidance 36 9

6/22/2018 73 yo F with IPF presents to ER Idiopathic Pulmonary Fibrosis with increased dyspnea. SaO2 58% 90% on 10LPM. Dry crackles at lung bases. Sputum gram stain • IPF is a specific form of chronic, progressive fibrotic interstitial lung is negative. CT shows new disease that occurs in older adults and areas of alveolar consolidation 26% is characterized by radiographic or superimposed on reticulation. pathologic usual interstitial pneumonia Which is the most appropriate without a secondary cause 11% next test? 5% • UIP pattern: peripheral basilar A. Bronchoscopy with BAL reticulation, traction bronchiectasis and honeycombing without other features B. Fungal serologies L y A s . d e . (i.e. ground glass, air trapping, etc) B . i i u g r t h o e s C. Right heart catheterization t l t i o e w – CTD, asbestosis, chronic HP, XRT w r h o e y t l s a l p c a o l a w D. Swallow study c g t r S s n a o u e h F h c n t h o g r 6/22/2018 Official ATS/ERS/JRS/ALAT Guidelines on Idiopathic Pulmonary Fibrosis . AJRCCM 2011;183:788 38 B i R IPF: Why is diagnosis important? IPF: Treatment Options • Median survival • IPF ~4 years Pirfenidone Nintedanib • Acute exacerbation rate 5-10% per year King, TE et al A phase 3 trial of pirfenidone in patients with IPF NEJM 2014 Ryerson CJ et al Predicting survival across interstitial lung disease: the ILD-GAP model Chest 2014 39 40 Richeldi, LR Efficacy and safety of nintedanib in IPF NEJM, 2014 10