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THE DIAGNOSIS OF IPF

THE DIAGNOSIS OF IPF. Steven A. Sahn, MD Professor of Medicine and Director Division of Pulmonary, Critical Care, Allergy and Sleep Medicine Medical University of South Carolina. Current Definition of IPF. Distinct chronic fibrosing interstitial pneumonia Unknown cause

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THE DIAGNOSIS OF IPF

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  1. THE DIAGNOSIS OF IPF Steven A. Sahn, MD Professor of Medicine and Director Division of Pulmonary, Critical Care, Allergy and Sleep Medicine Medical University of South Carolina

  2. Current Definition of IPF • Distinct chronic fibrosing interstitial pneumonia • Unknown cause • Limited to the lungs • Has typical HRCT findings • Associated with a histologic pattern of UIP ATS/ERS Consensus Statement. Am J Respir Crit Care Med. 2002;165:277-304.

  3. US Demographics • Incidence: > 30,000 patients/year • Prevalence: > 80,000 current patients • Age of onset: 40–70 years • Two-thirds > 60 years old at presentation • Males > females • Risk Factors • Familial • Smoking • Environment (eg, wood or metal dust) • Gastroesophageal reflux disease (GERD) • Infectious agents Demographics and Risk Factors for IPF ATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664. Raghu G, et al. Am J Respir Crit Care Med. 2006;174:810-816.

  4. Pulmonary Function Tests • Normal PFTs or resting ABG do not exclude IPF • Restriction (not always seen) • Reduced FVC and TLC • Normal or increased FEV1/FVC ratio • Impaired gas exchange • Decreased DLCO, PaO2 • Desaturation on exercise oximetry • Increased P(A-a)O2 gradient ATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664.

  5. Diagnosing Chronic Exertional Dyspnea Differential Diagnosis Others Neuromuscular Pulmonary Cardiac Vascular Anemia, Anxiety, Obesity, Deconditioning, hyperthyroidism Pulmonary Hypertension AVM NM disease, malnutrition, diaphragm dysfunction COPD, ILD, Asthma Cardiomyopathy, R-to-L Shunt YES No Basilar Velcro Crackles HRCT SCAN IPF Image courtesy of Steven A. Sahn, MD.

  6. Serologic Tests Can Help Exclude Other Conditions ESR ANA RF CK Aldolase Anti-myositis panel with Jo-1 antibody ENA panel p-ANCA Connective tissue diseases • Sarcoid ACE Hypersensitivity pneumonitis Hypersensitivity panel (exposure history) ATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664.

  7. Chest Radiograph in IPF Reduced lung volume Basal and peripheral reticulation Images courtesy of W. Richard Webb, MD.

  8. Early/Low Burden IPF HRCT Reticular opacities with a subpleural and basal predominance No honeycombing or traction bronchiectasis Image courtesy of W. Richard Webb, MD.

  9. Classic IPF HRCT Basal and subpleural predominance Reticular opacities Traction bronchiectasis Honeycombing Image courtesy of W. Richard Webb, MD.

  10. Advanced IPF HRCT Extensivehoneycombing Traction bronchiectasis Reticular opacities Basal and subpleural predominance Image courtesy of W. Richard Webb, MD.

  11. Diagnostic Criteria for IPF Without a Surgical Lung Biopsy All major criteria and at least 3 minor criteria must be present to increase the likelihood of an IPF diagnosis 1. Not included in current guidelines ATS/ERS. Am J Respir Crit Care Med. 2000;161:646-664.

  12. Radiologic Diagnosis Inconclusive Subpleural reticular opacities Both scans show subpleural reticulation. This appearance may represent early UIP/IPF or fibrotic NSIP. Biopsy is needed for their differentiation. Images courtesy of W. Richard Webb, MD.

  13. Video-Assisted Thoracic Surgery (VATS) • High diagnostic accuracy • Less morbidity and mortality than open lung biopsy • Ideal biopsy • Two or more surgical wedge biopsies with areas of normal lung from different sites of the lung • Samples 3–5 cm in length and 2–3 cm in depth • Outpatient thoracoscopic lung biopsy in patients with interstitial or focal lung disease • Diagnosis in 61/62 patients • 72% discharged within 8 hours • 22% discharged within 23 hours Rena O, et al. Eur J Cardiothorac Surg. 1999;16:624-627. Chang AC, et al. Ann Thorac Surg. 2002;74:1942-1946.

  14. Pathological Sections Demonstrating UIP b. Transition into uninvolved lung a. Peripheral accentuation of disease Fibrosis Normal lung Fibroblast focus Normal lung Normal lung d. High power image of fibroblastic foci c. Microscopic honeycombing Myofibroblasts Chronic inflammation Minimal chronic inflammation Minimal Chronic inflammation Mucus-filled cysts Courtesy of Kevin O. Leslie, MD. Courtesy of Kevin O. Leslie, MD. Pleura Images courtesy of Kevin O. Leslie, MD.

  15. Points to Remember • Typical clinical features: male > 50 years, smoker, insidious onset of dyspnea, non-productive cough and bibasilar Velcro crackles • Other diseases, such as CTD and sarcoidosis need to be excluded • Surgical lung biopsy necessary when typical clinical and HRCT findings of IPF not present • IPF: characteristic UIP histology enables definitive diagnosis when clinical/radiologic findings not conclusive

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