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The role of pirfenidone in the treatment of interstitial pneumonia with autoimmune features


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15

 

  1. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  2. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  3. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  4. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  5. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  6. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  7. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  8. Department of Radiology, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  9. Department of Radiology, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  10. Department of Rheumatology, South Campus, Ren Ji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
  11. Department of Rheumatology, South Campus, Ren Ji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
  12. Department of Pathology, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  13. Department of Pathology, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China.
  14. Department of Medical Statistics, Tongji University, School of Medicine, Shanghai, China.
  15. Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University, School of Medicine, Shanghai, China. liw2013@126.com

CER14480
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PMID: 33822701 [PubMed]

Received: 31/01/2021
Accepted : 15/03/2021
In Press: 30/03/2021

Abstract

OBJECTIVES:
No approved pharmacotherapies are available for patients with interstitial pneumonia with autoimmune features (IPAF). In the present work, we aimed to evaluate the efficacy and safety of pirfenidone for the treatment of IPAF.
METHODS:
A retrospective cohort study consisting of patients who met diagnostic criteria for IPAF was performed after a multidisciplinary review, and the patients receiving pirfenidone were compared with those in the non-pirfenidone group. The baseline data and diagnostic characteristics of patients were assessed. Pulmonary function and prednisone dose were analysed by a mix-effects model.
RESULTS:
A total of 184 patients, who met the diagnostic criteria of IPAF, were divided into two groups: pirfenidone group (n=81) and non-pirfenidone group (n=103). Patients in the pirfenidone group had a lower forced vital capacity (FVC%, p<0.001) and a lower diffusion capacity for carbon monoxide (DLCO%, p=0.003). The pirfenidone group exhibited a greater increase of FVC% at 6 (p=0.003), 12 (p=0.013), and 24 (p=0.003) months. After adjustment for sex, age, UIP pattern, baseline FVC% and DLCO%, patients in the pirfenidone group continued to show a greater improvement in FVC% (χ2(1)=4.59, p=0.032). Subgroup analysis identified superior therapeutic effects of pirfenidone in patients with dosage >600 mg/day (p=0.010) and medication course >12 months (p=0.007). Besides, the pirfenidone group had a lower prednisone dose than the non-pirfenidone group after 12 months of treatment (p=0.002). Moreover, 17 patients (19.32%) experienced side effects after taking pirfenidone, including one case of anaphylactic shock.
CONCLUSIONS:
Pirfenidone (600–1,800 mg/day) might help improve FVC, with an acceptable safety and tolerability profile in IPAF patients.

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