Steroids in idiopathic pulmonary fibrosis: A prospective assessment of adverse reactions, response to therapy, and survival

PURPOSE: We evaluated the risk and potential benefit of high-dose corticost eroid therapy in patients with idiopathic pulmonary fibrosis. SUBJECTS AND METHODS: We prospectively studied 41 patients with previously untreated, biopsy-proven idiopathic pulmonary fibrosis. Before treatment, w e calculated clinical, radiographic, and physiologic severity-of-illness sc ores for each patient. We scored high-resolution computerized tomographic ( CT) scans for ground glass and interstitial opacity. We determined the exte nt of cellular infiltration, interstitial fibrosis, desquamation, and granu lation in open lung biopsy samples. Patients-were monitored monthly for ste roid-related side effects, response to therapy at 3 months, and mortality. RESULTS: All patients experienced at least one steroid-induced side effect. Eleven (27%) patients were nonresponders, 11 (27%) were responders, and 19 (46%) remained stable. Of the 19 patients who died during a mean (+/- SD) follow-up of 3.3 +/- 2.3 years, 8 (42%) lost weight during the initial 3 mo nths of steroid therapy; only 3 (14%) of the 22 patients still living (P = 0.08) experienced weight loss. In a multivariate analysis, greater fibrosis (hazard ratio [HR] = 1.4 per unit increase; 95% confidence interval [CI]: 1.0 to 1.9; P = 0.03) and cellularity (RR = 1.9 per unit increase; 95% CI: 1.3 to 2.8; 3, P <0.001) in the biopsy sample and whether a patient was cla ssified as a responder (RR = 0.4 versus nonresponder; 95% CI: 0.2 to 1.0; P = 0.05) or stable (RR = 0.2 versus nonresponder; 95% CI: 0.1 to 0.6, P <0. 001) after steroid therapy were associated with mortality. CONCLUSION: Corticosteroid treatment for idiopathic pulmonary fibrosis is a ssociated with substantial morbidity. Patients who remain stable or respond to corticosteroid therapy have better survival than those who fail to resp ond. Whether this difference reflects an effect of treatment or less severe disease can be determined only in a randomized trial.