[Acute exacerbation of idiopathic pulmonary fibrosis]

Abstract

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.