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Interstitial Lung Disease for the PCP Jeff Swigris, DO, MS Associate Professor of Medicine Interstitial Lung Disease Program National Jewish Health Denver, Colorado
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swigrisj@njhealth.org
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Objectives Define the interstitium Define ILD Finding the cause Clinical presentation Therapy Define internist’s role
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Where is the interstitium?
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170,000-800,000 alveoli in here ~1-1.5cm
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Classification based on etiology ILD Exposure-related mold, bacteria, birds medications XRT dusts cigarette smoke Idiopathic Sarcoidosis IIP Genetic FPF CTD-related RA Systemic sclerosis PM/DM Sjögren’s syndrome MCTD UCTD SLE
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DAD DIP OP RB-ILD CFA IPF BOOP COP LIP NSIP UIP AIP Hamman-Rich BO HP UIPOB
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Idiopathic interstitial pneumonias (IIP) Idiopathic pulmonary fibrosis (IPF) Nonspecific interstitial pneumonia (NSIP) Cryptogenic organizing pneumonia (COP) (Idiopathic BOOP) Acute interstitial pneumonia (AIP) Desquamative interstitial pneumonia (DIP) Respiratory bronchiolitis-ILD (RB-ILD) Lymphoid interstitial pneumonia (LIP)
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Classification based on histology
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ILD Exposure-related mold, bacteria, birds medications XRT dusts cigarette smoke Idiopathic Sarcoidosis LAM IIP Genetic FPF Autoimmune-related RA Systemic sclerosis PM/DM Sjögren’s syndrome MCTD
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Nicholson et al. Am J Respir Crit Care Med 2000;162:2213-2217 Scar = bad prognosis Fibrosis Inflammation
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What type of fibrosis is the PCP most likely to see? ++++ Idiopathic pulmonary fibrosis (IPF) Aging population ++++ Connective tissue disease-related RA + Chronic hypersensitivity pneumonitis Organic exposure (M/M/B/B
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Making the diagnosis You have to be a detective History Exam Pulmonary physiology Radiography +/- surgical lung biopsy
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History: chief complaint Typically, ILD presents with: Dyspnea—subacute, insidious onset “I thought I was just…” Getting older 5# heavier Out of shape +/- dry cough Fatigue No wheeze, no chest pain
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History History Be a good detective Symptoms/existence of concurrent disease Patients may… 1. Have known CTD 2. Dyspnea from occult CTD-related ILD Family history Pulmonary fibrosis Rheumatologic illness
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History: exposures History: exposures Be a good detective Smoking PEARL IPF DIP, RB-ILD, PLCH Goodpasture’s
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History: exposures History: exposures Be a good detective Current or previous medications www.pneumotox.com www.pneumotox.com Chemotherapy Amiodarone Nitrofurantoin External beam radiation Current or previous recreational drug use Occupational, environmental, avocational PEARL
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History: exposures History: exposures Be a good detective Microbial agents M/M/B/B Hot tubs (indoor/enclosed) Basement shower Free-standing humidifiers Water damage to home Cooling systems (swamp cooler)
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History: exposures History: exposures Be a good detective Birds (proteins) Bloom on feathers Mucin in excrement Feather pillow/down comforter Fumes, dusts, gases Asbestos Beryllium
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History: connective tissue diseases RA Symmetric arthritis/small joints Morning stiffness Subcutaneous nodules Smoker PEARL
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History: connective tissue diseases SSc Raynauds After 40 y.o. in FEMALE After 30 y.o. in MALE Esophageal dysmotility Skin tightening PEARL
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History: connective tissue diseases Sjögren’s Syndrome Dry eyes/mouth Dental caries
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History: connective tissue diseases PM/DM Proximal muscle weakness Rashes Rough skin on the hands
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Physical Exam
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Physical examination Physical examination You’re still a detective Skin Rash Purupura Telangiectasia Nodules Calcinosis
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Physical examination Nails Clubbing COPD no clubbing PEARL
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Nailfold capillaroscopy Normal Abnormal Fischer et al. Chest. In press
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Physical examination Chest Velcro crackles are NEVER normal Must listen here PEARL
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Laboratory ANA—the pattern matters Nucleolar ANA any titer – TO RHEUM SSA is a myositis associated ab (ANA -) ACE level non-specific Don’t order it HP panels unhelpful Precipitating IgG to organic antigens Don’t order them PEARLS
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Laboratory Isolated high MCV Methotrexate Azathioprine ??? Telomerase abnormality Elevated MCV History of bone marrow irregularities Premature graying Cryptogenic cirrhosis Pulmonary fibrosis PEARLS
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Pulmonary physiology Pulmonary function testing Gas exchange
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Pulmonary function testing Lung volumes Spirometry DLCO ABG
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Patients with ILD have Restrictive Physiology Low static lung volumes Low forced volumes Low FVC Low FEV1 Normal FEV1/FVC
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Volumes may be normal if… + …but the DLCO will be very low
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Impaired Gas Exchange SpO2 at rest is unhelpful Exercise oximetry Never normal to desaturate 6-minute walk test PEARL
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Radiology: diagnosing ILD “ILD protocol” HRCT No IV contrast Supine and prone Inspiratory and expiratory images Reconstruction algorithm — 1-1.5mm thick
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HRCT Terminology Opacities Lines (reticular) Dots or Circles (nodules) Patches Attenuation (shade of gray) Consolidation – obscures underlying vessels Ground glass – does not obscure underlying vessels
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Lower zone Peripheral/subpleural Reticular opacities Traction bronchiectasis Interlobular septal thickening
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Honeycombing
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Ground glass opacities
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Lung biopsy Transbronchial biopsy Sarcoidosis Lymphangitic carcinomatosis Subacute HP Surgical Thorascopic Usually not if CTD-related
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Putting it all Together History Exam Labs ANA, RF, anti-CCP Physiology Full PFTs Gas exchange 6MWT Radiology HRCT Pathology Integrate to get “summary diagnosis”
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Therapy for ILD Not all patients require therapy General: treat clinically significant, progressive dz All therapeutic regimens require monitoring Glucocorticoids may be the mainstay Steroid-sparing / immune-suppressing / immunomodulatory / cytotoxic agents Nuance
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STABILITY = SUCCESS I don’t want my patients ILD leaving clinic thinking they don’t have a serious condition I don’t want my patients with ILD leaving clinic thinking they should go home, sit on their couch and die
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Gauging Response Q 3mos visits to pulm Subjective Symptoms FVC DLCO 6MWT Not HRCT unless scenario mandates
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Internist: before ILD dx Thorough history and examination Order HRCT Order serologies ANA with pattern and ENA panel RF/anti-CCP Order PFTs/6MWT/HRCT Refer: ILD on HRCT
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Internist: after ILD dx Monitor for side effects of therapy Glucocorticoids Weight Sugar BP Eyes Bones Be on the lookout for infection Monitor need for oxygen Communicate with patient Mood: therapy needed? End-of-life discussions
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Internist: after ILD dx Refer to pulmonary rehabilitation Vaccines Sunscreen for all on immunosuppressive Tx Monthly labs for all on immunosuppressive Tx
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Five Main Points You will see ILD — be a detective Velcro crackles never normal — get HRCT Surgical lung biopsy often needed to make a confident diagnosis All patients and most therapies require monitoring—the internist is vital here
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