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Interstitial Lung Disease for the PCP

Interstitial Lung Disease for the PCP. Jeff Swigris, DO, MS Associate Professor of Medicine Interstitial Lung Disease Program National Jewish Health Denver, Colorado. swigrisj@njhealth.org. Objectives. Define the interstitium Define ILD Finding the cause Clinical presentation Therapy

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Interstitial Lung Disease for the PCP

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  1. Interstitial Lung Disease for the PCP Jeff Swigris, DO, MS Associate Professor of Medicine Interstitial Lung Disease Program National Jewish Health Denver, Colorado

  2. swigrisj@njhealth.org

  3. Objectives • Define the interstitium • Define ILD • Finding the cause • Clinical presentation • Therapy • Define internist’s role

  4. Where is the interstitium?

  5. 170,000-800,000 alveoli in here ~1-1.5cm ~1-1.5cm

  6. Classification based on etiology ILD GeneticFPF Exposure-relatedmold, bacteria, birds medications XRT dusts cigarette smoke Idiopathic Sarcoidosis IIP CTD-relatedRA Systemic sclerosis PM/DM Sjögren’s syndrome MCTD UCTD SLE

  7. BOOP DAD DIP BO OB UIP HP NSIP LIP COP Hamman-Rich AIP RB-ILD IPF UIP CFA OP

  8. 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)

  9. Classification based on histology

  10. ILD GeneticFPF Exposure-relatedmold, bacteria, birds medications XRT dusts cigarette smoke Idiopathic Sarcoidosis LAM IIP Autoimmune-relatedRA Systemic sclerosis PM/DM Sjögren’s syndrome MCTD

  11. Scar = bad prognosis Inflammation Fibrosis Nicholson et al. Am J Respir Crit Care Med 2000;162:2213-2217

  12. 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

  13. Making the diagnosis You have to be a detective • History • Exam • Pulmonary physiology • Radiography • +/- surgical lung biopsy

  14. 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

  15. HistoryBe 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

  16. History: exposuresBe a good detective • Smoking PEARL • IPF • DIP, RB-ILD, PLCH • Goodpasture’s

  17. History: exposuresBe a good detective • Current or previous medications • www.pneumotox.com • Chemotherapy • Amiodarone • Nitrofurantoin • External beam radiation • Current or previous recreational drug use • Occupational, environmental, avocational PEARL

  18. History: exposuresBe 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)

  19. History: exposuresBe a good detective • Birds (proteins) • Bloom on feathers • Mucin in excrement • Feather pillow/down comforter • Fumes, dusts, gases • Asbestos • Beryllium

  20. History: connective tissue diseases • RA • Symmetric arthritis/small joints • Morning stiffness • Subcutaneous nodules • Smoker PEARL

  21. History: connective tissue diseases • SSc • Raynauds • After 40 y.o. in FEMALE • After 30 y.o. in MALE • Esophageal dysmotility • Skin tightening PEARL

  22. History: connective tissue diseases • Sjögren’s Syndrome • Dry eyes/mouth • Dental caries

  23. History: connective tissue diseases • PM/DM • Proximal muscle weakness • Rashes • Rough skin on the hands

  24. Physical Exam

  25. Physical examinationYou’re still a detective • Skin • Rash • Purupura • Telangiectasia • Nodules • Calcinosis

  26. Physical examination • Nails • Clubbing • COPD no clubbing PEARL

  27. Nailfold capillaroscopy Abnormal Normal Fischer et al. Chest. In press

  28. Physical examination • Chest • Velcro crackles are NEVER normal PEARL Must listen here

  29. Laboratory PEARLS • 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

  30. Laboratory PEARLS • Isolated high MCV • Methotrexate • Azathioprine • ??? Telomerase abnormality • Elevated MCV • History of bone marrow irregularities • Premature graying • Cryptogenic cirrhosis • Pulmonary fibrosis

  31. Pulmonary physiology • Pulmonary function testing • Gas exchange

  32. Pulmonary function testing • Lung volumes • Spirometry • DLCO • ABG

  33. Patients with ILD have Restrictive Physiology • Low static lung volumes • Low forced volumes • Low FVC • Low FEV1 • Normal FEV1/FVC

  34. Volumes may be normal if… + …but the DLCO will be very low

  35. Impaired Gas Exchange • SpO2 at rest is unhelpful • Exercise oximetry • Never normal to desaturate • 6-minute walk test PEARL

  36. Radiology: diagnosing ILD • “ILD protocol” HRCT • No IV contrast • Supine and prone • Inspiratory and expiratory images • Reconstruction algorithm — 1-1.5mm thick

  37. 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

  38. Interlobular septal thickening Traction bronchiectasis Reticular opacities Peripheral/subpleural Lower zone

  39. Honeycombing

  40. Ground glass opacities

  41. Lung biopsy • Transbronchial biopsy • Sarcoidosis • Lymphangitic carcinomatosis • Subacute HP • Surgical • Thorascopic • Usually not if CTD-related

  42. 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”

  43. 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

  44. 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

  45. Gauging Response • Q 3mos visits to pulm • Subjective • Symptoms • FVC • DLCO • 6MWT • Not HRCT unless scenario mandates

  46. 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

  47. 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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