Pulmonary fibrosis

(Fr: fibrose pulmonaire). Background rate of idiopathic IPF at PMID 25363218. Imaging criteria at PMID 23672718. The diagnosis of interstitial pulmonary fibrosis is raised in the presence of pulmonary opacities and architectural distortion on imaging (HRCT), restrictive lung dysfunction, low KCO, and hypoxemia obviating the need for a lung biopsy. Presentation can be chronic, subacute or rapidly progressive. Generally, the condition develops insidiously with dyspnea and bibasilar or diffuse reticular or streaky opacities. Honeycombing can develop with time in longterm survivors. DI-fibrosis may follow a classic episode of chemotherapy- or amiodarone-induced pulmonary toxicity. Separation of DI-fibrosis from idiopathic or CTD (viz. RA or scleroderma)-related fibrosis can be arduous (PMID 23791462). Some patients exacerbate and progress to ARDS. DI-fibrosis may develop late after completion of therapy with chemo agents, irradiation or amiodarone, with no evidence of toxicity during treatment or in the meantime, although DI-fibrosis rarely occurs as a complication of patterns Ia, Ib, Ic. Oxygen and irradiation can trigger the onset or exacerbate DI-fibrosis. When DI-fibrosis develops during treatment with the offending agent, drug withdrawal may translate into some improvement. The effect of costicosteroid therapy is limited and unpredictable

Last update : 17/11/2014

Causative drugs