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

Ting Li1, Li Guo1, Zhiwei Chen1

  • 1Department of Rheumatology, Ren Ji Hospital South Campus, School of Medicine, Shanghai JiaoTong University, Shanghai, 200001, China.

Scientific Reports
|September 13, 2016
PubMed

Insights

Pirfenidone may improve survival in subacute interstitial lung disease (ILD) linked to clinically amyopathic dermatomyositis (CADM). This treatment showed a higher survival rate in subacute CADM-ILD patients compared to controls.

Area of Science:

  • Pulmonology
  • Rheumatology
  • Pharmacology

Background:

  • Interstitial lung disease (ILD) can rapidly progress in patients with clinically amyopathic dermatomyositis (CADM).
  • Evaluating novel therapeutic strategies for CADM-ILD is crucial due to its poor prognosis.

Purpose of the Study:

  • To assess the efficacy of pirfenidone as an add-on therapy for patients with rapidly progressive ILD (RPILD) associated with CADM.
  • To determine the impact of pirfenidone on mortality and survival rates in this patient population.

Main Methods:

  • An open-label, prospective study involving 30 patients with CADM-RPILD treated with pirfenidone and conventional therapy.
  • A matched retrospective control group of 27 patients with CADM-RPILD not receiving pirfenidone.
  • Subgroup analysis based on disease duration: acute ILD (<3 months) and subacute ILD (3-6 months).

Main Results:

  • The pirfenidone add-on group showed a trend towards lower overall mortality (36.7% vs. 51.9%).
  • Pirfenidone did not significantly impact survival in acute ILD patients (50% vs. 50%).
  • Pirfenidone significantly improved survival in subacute ILD patients (90% vs. 44.4%, p=0.0450).

Conclusions:

  • Pirfenidone add-on therapy may enhance prognosis in patients with subacute ILD related to CADM.
  • Further research is warranted to confirm these findings and optimize treatment strategies for CADM-ILD.

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