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Rapid disease progression is an important cause of death in IPF
  1. S H Siddiqui
  1. SPR/Clinical Research Fellow in Respiratory Medicine, Glenfield Hospital, Leicester, UK;

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The authors examined data generated from the placebo arm of a large multicentre double blind trial of interferon-γ1b in patients with mild to moderate idiopathic pulmonary fibrosis (IPF), collected over a median period of 76 weeks at 12 weekly intervals.

IPF was diagnosed according to the ATS criteria, by surgical lung biopsy or established HRCT criteria in 168 patients (mean age 64 years, 66% male, 86% white, 91% never or ex-smokers). At entry most patients (82%) were receiving oral corticosteroids in a dose of less than 15 mg daily, the mean duration since diagnosis was over 1 year, and the mean (SD) carbon monoxide transfer factor (Tlco) was 36.8 (10.6)%. Patients who survived to week 72 (78.6%) had minimal deterioration in physiology and dyspnoea (FVC, Tlco, A–a gradient, transitional dyspnoea index). Thirty six patients (21.4%) died during the observation period. In those who died of an IPF related death (32/36), nearly 50% died acutely (<4 weeks). Progression of IPF and pneumonia were the most common causes (63% and 13%, respectively). Patients who died had a progressive decline in measures of physiology and dyspnoea throughout the observation period. More than one third of patients were admitted to hospital, often frequently (1.67 admissions/patient), 23% for a respiratory disorder with a mean length of stay of 14.3 days.

This study provides novel insights into the natural history of IPF. In particular, a large percentage of deaths had an acute onset, suggesting that accelerated IPF may be more common than was once perceived. Hospitalisation occurred commonly and frequently. A substantial proportion of patients had a minimal decline in conventional measures of lung function and dyspnoea, highlighting disease heterogeneity and posing challenges for measuring efficacy of new pharmaceutical compounds. Further prospective studies are required to elaborate on these findings.