Research Article: Cumulative Incidence and Predictors of Progression in Corticosteroid-Naïve Patients with Sarcoidosis

Date Published: November 17, 2015

Publisher: Public Library of Science

Author(s): Yusuke Inoue, Naoki Inui, Dai Hashimoto, Noriyuki Enomoto, Tomoyuki Fujisawa, Yutaro Nakamura, Takafumi Suda, Antje Prasse.


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.

This study aimed to clarify the cumulative incidence and identify risk factors for disease progression in corticosteroid-naïve sarcoidosis patients.

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.

The median follow-up duration was 7.7 years (interquartile range, 4.7–13.6 years). Thirty-two (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.

In corticosteroid-naïve 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.

Partial Text

Sarcoidosis is a systemic granulomatous disorder of unknown cause. Sarcoidosis affects multiple organs, and patients with sarcoidosis have a diverse clinical course and prognosis. Spontaneous remission occurs in two thirds of cases [1]. On the other hand, disease progression occurs in some fraction of cases of sarcoidosis. The prevalence of deterioration of pulmonary sarcoidosis widely ranges from 13% to 75% of patients [2–5]. Although assessment of disease progression in sarcoidosis is a major problem, in which patients and in what manner it occurs are not fully understood. Furthermore, few data are available concerning the long-term course of sarcoidosis because most studies assessed the natural history of this disease 2–5 years after diagnosis [3, 4, 6–8].

To the best of our knowledge, this study is the first to investigate the cumulative incidence of disease progression and new organ involvement in corticosteroid-naïve patients with sarcoidosis. The risks of initial deterioration at 0–5 and 5–10 years after diagnosis were almost equivalent. We found that the number of involved organs at the time of diagnosis was an independent factor to predict disease progression.