Acute exacerbation of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease. Its natural history may have episodes of acute exacerbation (AE-IPF), whose best accepted definition would be a clinically significant acute worsening, without identified cause, in patients with an underlying IPF. The incidence of AE-IPF ranges from 5%-19% of patients per year. It is estimated that acute deterioration of the respiratory status occurred in 47% of these patients before death. A rapidly progressive dyspnoea is the most prominent symptom, with associated cough, fever and flu-like symptoms. The diagnosis is strengthened by the presence of leukocytosis on the blood count, neutrophilia on the bronchoalveolar lavage, and development of new diffuse bilateral ground-glass opacities superimposed on the pre-existing radiographic findings. The major histological finding is diffuse alveolar damage. Methylprednisolone seems to be the best therapeutic option, although the treatment response is usually poor. (C) 2009 Elsevier Espana, S.L. All rights reserved.