Cumulative incidence and predictors of progression in corticosteroid-naïve patients with sarcoidosis

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? 2015 Inoue et al.This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Background Assessment of the clinical course of sarcoidosis requires long-term observation. However, the appropriate period of follow-up for sarcoidosis remains unclear, especially in patients without indication of corticosteroid therapy at the time of diagnosis. Objective This study aimed to clarify the cumulative incidence and identify risk factors for disease progression in corticosteroid-na?ve sarcoidosis patients. Methods The clinical courses of 150 Japanese patients with sarcoidosis, who were followed for more than 2 years and had no indication for corticosteroid therapy at diagnosis, were retrospectively reviewed. Disease progression was defined as worsening of pulmonary sarcoidosis, development of new organ involvement, or extrapulmonary organ damage. The cumulative incidence of progression was estimated by generating a cumulative incidence curve with the Fine and Gray method. Results The median follow-up duration was 7.7 years (interquartile range, 4.7-13.6 years). Thirtytwo (21%) patients experienced disease progression. New organ involvement appeared in 16 patients (11%). The 6-month, and 1-, 5-, 10-, and 15-year cumulative incidence of progression was 2%, 5%, 15%, 28%, and 31%, respectively. The number of organs involved at diagnosis was an independent predictor for progression with a multifactorial adjusted hazard ratio of 1.71 (95% confidence interval, 1.11-2.62). The optimal cut-off of the number of organs involved at diagnosis to identify future progression was three. Conclusions In corticosteroid-na?ve sarcoidosis patients, the risks of disease progression are comparable from 0-5 years and 5-10 years after diagnosis. The number of organs involved at diagnosis is a useful predictor for progression of sarcoidosis.




Inoue, Y., Inui, N., Hashimoto, D., Enomoto, N., Fujisawa, T., Nakamura, Y., & Suda, T. (2015). Cumulative incidence and predictors of progression in corticosteroid-naïve patients with sarcoidosis. PLoS ONE, 10(11).

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