IPF Rx MD - MD Seminar Management of IPF Idiopathic...

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Unformatted text preview: MD Seminar Management of IPF Idiopathic pulmonary fibrosis z Also known as CFA { Chronic scarring illness limited to lungs & characterized by z Progressive dyspnea z Restrictive pulmonary physiology z Radiographically diffuse lung disease z Occurs in adults, manifesting beyond 5 th and 6 th decade z Until recently, diagnosis and management was difficult because { Vague case definition { Clinical features overlapped with other chronic interstitial pneumonias Epidemiology z IPF is most common diagnosis among all IIPs z Usually affects older adults and men z Risk factors { Familial IPF- 3% of cases, suggests genetic predisposition may interact with environmental factors { Environmental factors Smoking Inhalation of organic and inorganic dust Exposure to dust ( pinewood) Use of tricyclic antidepressants Gastroesophageal reflux { No connection between IPF and an infective agent Pathophysiology z Current hypothesis Recurrent, unknown injury (extrinsic or intrinsic) To distal pulmonary parenchyma Repeated epithelial injury Abnormal wound healing, Mediated by fibroblasts and fibroblast-like cells Or myofibroblasts Progressive fibrosis Natural history of IPF z Fatal illness { progressive impairment of lung compliance and gas exchange { Inexorable course { usually death because of hypoxic respiratory failure, associated with pulmonary hypertension & cor pulmonale { Increased risk of bronchogenic carcinoma z Median survival 80 months from the time of onset of symptoms (King et al.) z Acute exacerbations may occur in absence of infection { Fulminant decline in oxygenation { Rapidly progressive respiratory failure Diagnosis z Clinical evaluation- based on CRP { History z subacute course of progressive symptoms ( SOB & Cough) over months to years z Past, family and environmental history to rule out other types of ILDs ( HP, occupational ILD, CT-ILD) { Examination z Fine end inspiratory crackles at lung bases z Pulmonary hypertension & right heart failure late in disease z Clubbing in majority z Radiological { CXR- Useful initial visual perception of overall lung involvement z Low lung volumes z Bilateral reticular opacities- most severely involving periphery and bases of lungs z Features of heart failure z Features are not specific but demonstrate disease extent & progression & cardiopulmonary status {...
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IPF Rx MD - MD Seminar Management of IPF Idiopathic...

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