Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

Ting Li, Li Guo, Zhiwei Chen, Liyang Gu, Fangfang Sun, Xiaoming Tan, Sheng Chen, Xiaodong Wang, Shuang Ye, Ting Li, Li Guo, Zhiwei Chen, Liyang Gu, Fangfang Sun, Xiaoming Tan, Sheng Chen, Xiaodong Wang, Shuang Ye

Abstract

To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3-6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.

Figures

Figure 1. The survival curves of the…
Figure 1. The survival curves of the pirfenidone add-on group and the control group.
(A) Survival analysis of all patients showed that the pirfenidone add-on group had fewer deaths than the control group, but the difference was not significant. (B) The pirfenidone add-on had no impact on the survival of patients with acute ILD (disease duration <3 months). (C) The pirfenidone add-on led to a significantly higher survival rate in subacute ILD patients (disease duration 3–6 months) compared with the control subgroup.
Figure 2. The overall HRCT score at…
Figure 2. The overall HRCT score at baseline and its changes at 6 months among survivors.
The baseline level did not significantly differ between the pirfenidone and control groups or the deceased and survivor groups.

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