XXIV - Interstitial lung disease - Pulmonary infiltrates - Pulmonary fibrosis
Pulmonary fibrosis
Last update 20/09/2020
(Fr: fibrose pulmonaire). The background incidence of idiopathic pulmonary fibrosis (IPF) has to be taken into account for causality assessment. Imaging at PMID 23672718. The diagnosis of interstitial pulmonary fibrosis�is raised in the presence of linear pulmonary opacities and architectural distortion on imaging incl. 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).�Patients may 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. DI-fibrosis rarely occurs as a complication of patterns Ia, b or c. Oxygen and irradiation can trigger the onset of or exacerbate DI-fibrosis. When DI-fibrosis develops during treatment with the offending agent, drug withdrawal may translate into some improvement. Costicosteroid therapy is indicated, but the results are unpredictable
