Idiopathic Pulmonary Fibrosis - Pulmonary Medicine - Lecture Slides, Slides of Pneumology

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Idiopathic pulmonary fibrosis Also known as CFA Chronic scarring illness limited to lungs & characterized by Progressive dyspnea Restrictive pulmonary physiology Radiographically diffuse lung disease Occurs in adults, manifesting beyond 5th and 6th decade Until recently, diagnosis and management was difficult because Vague case definition Clinical features overlapped with other chronic interstitial pneumonias Docsity.com Epidemiology IPF is most common diagnosis among all IIPs Usually affects older adults and men 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 Docsity.com Diagnosis Clinical evaluation- based on CRP History – subacute course of progressive symptoms ( SOB & Cough) over months to years Past, family and environmental history to rule out other types of ILDs ( HP, occupational ILD, CT-ILD) Examination – Fine end inspiratory crackles at lung bases Pulmonary hypertension & right heart failure late in disease Clubbing in majority Docsity.com Radiological – CXR- Useful initial visual perception of overall lung involvement Low lung volumes Bilateral reticular opacities- most severely involving periphery and bases of lungs Features of heart failure Features are not specific but demonstrate disease extent & progression & cardiopulmonary status Docsity.com HRCT- necessary step in diagnostic evaluation First may illustrate characteristic features of UIP- • patchy, predominantly peripheral, sub pleural, bibasal reticular abnormalities • Traction bronchiectasis &/or sub pleural honeycombing • May enable clinical diagnosis without need for biopsy Secondly • clinical diagnosis of new onset IPF can be made accurately if classic clinical & HRCT features are present • If typical findings not present may identify other disease processes Thirdly • May assist in decision of whether or not to biopsy & the sample site(s) • If extensive fibrosis & honeycombing present, disease likely is end stage Docsity.com Treatment Conventional therapy Corticosteroids alone- ineffective and associated with significant side effects Corticosteroids + immunosuppressives Limited efficacy in slowing the progressive deterioration in lung function Slightly superior to corticosteroids alone, when initiated for new onset IPF Low dose prednisone + azathioprine- frequently used initial treatment in IPF Alternative -Prednisone + cyclophosphamide Docsity.com Newer therapies- required because of ineffectiveness and side effects of conventional therapy Immuno modulators Interferon γ - inhibitory effect on fibroblasts Antifibrotic agents Colchicine D-penicillamine Pirfenidone ACE inhibitors Statins Anti-oxidants N-acetylcysteine Docsity.com Endothelial receptor-1 antagonist Bosentan Tumor necrosis factor-α blocker Etanercept Imatinib mesylate - cAbl tyrosine kinase inhibitor + PDGF receptor antgonist CTGF blocker FG-3019 Rapamycin Antileukotriene drugs Zileuton Docsity.com Approach to treatment Therapy is not indicated for all patients Age >70 years Extreme obesity Concomitant major illness Severe impairment in pulmonary function End stage honeycomb on radiology Treatment should be started At the first identification of clinical or physiological evidence of impairment of lung function Early in the course of the disease before irreversible fibrosis has developed Docsity.com Combination therapy is a reasonable approach Corticosteroid (prednisone or equivalent) 0.5mg/kg/day orally Χ 4 weeks 0.25 mg/kg/day Χ 8 weeks 0.125 mg/kg/day as initial therapy Azathioprine at 2-3 mg/kg/day to maximum dose of 150mg/day orally Begin at 25-50 mg/day Increase by 25 mg increments every 7-14 days Or Cyclophosphamide Docsity.com Length of therapy – objective response may take >3 months of therapy Combined therapy should be continued for at least 6 months in absence of complications Monitoring of therapy At 6 month If patient worse – therapy stop or changed If patient improved or stable - therapy continued at same doses At 12 months If patient worse - therapy stop or changed If patient improved or stable - therapy continued at same doses At 18 month or more Therapy continued indefinitely only in individuals with evidence of continued improvement or stabilization Docsity.com Failure to respond to therapy- (after 6 month of therapy) Increase in symptoms Increase in opacities, honeycombing, PHT Deterioration in lung function ≥10% decrease in TLC or VC (or ≥200-ml change) ≥15% decrease in single breath DLco Worsening of O2 saturation Docsity.com Monitoring for adverse effects Corticosteroids Azathioprine & cyclophosphamide Hematological Hepatic Oncogenic potential Hemorrhagic cystitis Also monitor for Opportunistic lung infection Pneumonia Pulmonary emboli Secondary pulmonary hypertension Heart failure Neoplasm Docsity.com Lung transplantation Considered for progressive physiologic deterioration despite therapy and who meet established criteria Severe functional impairment Oxygen dependency Deteriorating course <60 year age Early listing important because long waiting period 5 year survival- 50-60% Docsity.com