• Vincent Cottin.
• Rev Prat. 2014 Sep 1; 64 (7): 923-8, 930-2.

AbstractIdiopathic pulmonary fibrosis is a chronic disease, which affects more frequently subjects older than 60 years, males, and smokers or ex-smokers. The diagnosis is based on a pattern of usual interstitial pneumonia at high resolution computed tomography of the chest and/or at the video- assisted thoracic surgical lung biopsy, and by multidisciplinary discussion in a specialized center, after ruling out possible causes and specific contexts (as connective tissue disease). The disease progresses inexorably to chronic respiratory insufficiency, occasionally with episodes of acute exacerbation, and death after a median of 3 years. Treatment with pirfenidone in patients with mild to moderate disease slows the progression of disease, and reduces the risk of death at one year, with skin and gastro-intestinal adverse events. Drug therapy should be part of a global care management. Several approaches are considered for an earlier diagnosis and treatment.

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