To the Editors:
Acute exacerbations (AEs) are now recognised as a frequent and severe complication of idiopathic pulmonary fibrosis (IPF). In a large series, 1- and 3-yr incidences were 14.2% and 20.7%, respectively [1]. New diagnostic criteria were published in 2007 [2]. The pathogenesis of these episodes remains unknown, although invasive procedures have been described as possible triggers. Prognosis is poor, with a short-term mortality rate of 45–85%. There is no consensus regarding treatment, as no published study has compared the efficiency of different treatment regimens, and treatment often differs between patients within a given study [1–4]. Since 2005, exacerbations of IPF identified in our referral centre (Centre Hospitalier Régional de Lille, Lille, France) have been treated with pulses of methylprednisolone followed by pulses of cyclophosphamide [5]. The main goal of the present retrospective study was to evaluate the mortality of exacerbations of IPF treated with this regimen.
Following admission for aggravation of dyspnoea, a series of tests were run to determine diagnosis and functional impairment. When an exacerbation of IPF was diagnosed, patients were treated with a methylprednisolone pulse (1,000 mg) at days 1–3 and on day 4 placed on an escalating regimen of cyclophosphamide with an initial dose of 500 mg intravenously [5]. The dose of cyclophosphamide was increased by 200 mg every 2 weeks, provided the total white blood cell count remained at >3,000 cells·mm−3. The maximum single administered dose was 1,500 mg of cyclophosphamide. The diagnosis of IPF was reassessed for the purpose of this study …
