Hospital-based historical cohort study of 234 histologically proven Japanese patients with IPF.

Abstract

Variable clinical courses have been recognized in patients with idiopathic pulmonary fibrosis (IPF), but the disease is known to have a fundamentally unfavorable outcome. In addition, we have yet to fully understand whether the regional or population differences are negligible or not. Therefore, we analyzed the clinical outcomes in Japanese IPF patients in a hospital-based historical cohort study. A questionnaire was used to collect records for retrospective analysis of the IPF patients. We analyzed data collected from 51 hospitals and clinics in Japan on 234 Japanese IPF patients (males 170, females 64) with IPF who had been followed up after diagnosis. 1. Five and ten years after the detection of exertional dyspnea, the overall survival rates were 41.0% and 20.1%, respectively. A higher age at the time of detection (over 59) was related to a decrease in survival rates. 2. The incidence of complications was no higher in the corticosteroid-treated cases than in the untreated cases. The effects of corticosteroid treatment on survival was not confirmed in this type of study. The survival rates of histologically proven Japanese patients with IPF were similar to the rates previously reported in different populations. Age at the detection of exertional dyspnea was critical in terms of the survival rate.