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Medicine (Baltimore). 2014 Nov;93(26):e175. doi: 10.1097/MD.0000000000000175.

Predictors of mortality in patients with interstitial lung disease treated with corticosteroids: results from a cohort study.

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From the Japanese National Hospital Organization (NHO)-EBM study group for Adverse Effects of Corticosteroid therapy (J-NHOSAC), Meguro, Tokyo (KM, TA, ES, TM, HT, YK, RM, SM, SO, SY, YS, FO, MK, HF, TM, SB, ST); and Department of Rheumatology and General Internal Medicine Nagasaki Medical Center, Omura, Nagasaki, Japan (KM, YJ, YI, NI, CK).


Interstitial lung disease (ILD) has a heterogeneous clinical presentation and establishing prognosis for these patients is challenging. We investigated the clinical characteristics and outcome of patients with idiopathic interstitial pneumonias (IIPs) and patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). We conducted a multicenter prospective study on 104 patients diagnosed with IIPs and 29 patients diagnosed with CTD-ILD, which were newly diagnosed and treated with corticosteroids initially. We compared the clinical characteristics, high-resolution computed tomography (HRCT) imaging date, and outcomes. Cox proportional hazard regression analysis was used to identify variables with increased risk of death. Survival was analyzed according to the Kaplan-Meier method and was assessed with the log-rank test. Of 133 patients with IIPs (n = 104) or CTD-ILD (n = 29), 44 patients died during the follow-up period (mean: 1.6 ± 0.78 years). Patients with IIPs seemed to be associated with worse survival compared with those with CTD-ILD; however, this difference was not significant (log-rank test, P = 0.084). Significant predictors for mortality in patients with IIPs at baseline were lower for performance status and definite usual interstitial pattern (UIP) on HRCT. Patients with UIP experienced worse survival than those with non-UIP. A definite UIP on HRCT and lower baseline performance status have important prognostic implications in patients with IIPs.

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