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Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

 Myung Jin Song  ;  Sang Hoon Lee  ;  Ji Ye Jung  ;  Young Ae Kang  ;  Moo Suk Park  ;  Young Sam Kim  ;  Joon Chang  ;  Song Yee Kim 
 BMC PULMONARY MEDICINE, Vol.21(1) : 145, 2021-05 
Journal Title
Issue Date
Autoantibodies ; Idiopathic pulmonary fibrosis ; Pirfenidone
Background: Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear.

Methods: We retrospectively reviewed the medical records of patients with IPF treated at a tertiary care hospital in South Korea. The autoantibody status was defined as positive if we detected autoantibodies meeting the serological domain criteria for interstitial pneumonia with autoimmune features or anti-neutrophil cytoplasmic antibodies.

Results: We included 142 patients with IPF treated with pirfenidone for over six months (93 were autoantibody-positive and 49 were autoantibody-negative). The mean age was 69.5 ± 7.3 years, and 77.5% of the patients were male. The adjusted mean changes over one year were - 34.4 and - 112.2 mL (p = 0.168) in forced vital capacity (FVC), and - 0.53 and - 0.72 mL/mmHg/min (p = 0.356) in the lungs diffusion capacity for carbon monoxide (DLCO) in the autoantibody-negative and autoantibody-positive groups, respectively.

Conclusions: Reductions in FVC and DLCO were similar in autoantibody-positive and autoantibody-negative patients with IPF treated with pirfenidone. Pirfenidone is effective in attenuating the progression of IPF, irrespective of the autoantibody status.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Kang, Young Ae(강영애) ORCID logo https://orcid.org/0000-0002-7783-5271
Kim, Song Yee(김송이) ORCID logo https://orcid.org/0000-0001-8627-486X
Kim, Young Sam(김영삼) ORCID logo https://orcid.org/0000-0001-9656-8482
Park, Moo Suk(박무석) ORCID logo https://orcid.org/0000-0003-0820-7615
Lee, Sang Hoon(이상훈) ORCID logo https://orcid.org/0000-0002-7706-5318
Chang, Joon(장준) ORCID logo https://orcid.org/0000-0003-4542-6841
Jung, Ji Ye(정지예) ORCID logo https://orcid.org/0000-0003-1589-4142
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