PMID- 33822701
OWN - NLM
STAT- MEDLINE
DCOM- 20220331
LR  - 20220401
IS  - 0392-856X (Print)
IS  - 0392-856X (Linking)
VI  - 40
IP  - 3
DP  - 2022 Mar
TI  - The role of pirfenidone in the treatment of interstitial pneumonia with 
      autoimmune features.
PG  - 560-567
LID - 10.55563/clinexprheumatol/off5n7 [doi]
AB  - 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% (chi2(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.
FAU - Chen, Tao
AU  - Chen T
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China.
FAU - Li, Qiu-Hong
AU  - Li QH
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China.
FAU - Zhang, Yuan
AU  - Zhang Y
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China.
FAU - Yin, Cheng-Sheng
AU  - Yin CS
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China.
FAU - Weng, Dong
AU  - Weng D
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China.
FAU - Zhou, Ying
AU  - Zhou Y
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China.
FAU - Hu, Yang
AU  - Hu Y
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China.
FAU - Shi, Jing-Yun
AU  - Shi JY
AD  - Department of Radiology, Shanghai Pulmonary Hospital, Tongji University, School 
      of Medicine, Shanghai, China.
FAU - Chen, Ya-Nan
AU  - Chen YN
AD  - Department of Radiology, Shanghai Pulmonary Hospital, Tongji University, School 
      of Medicine, Shanghai, China.
FAU - Ye, Shuang
AU  - Ye S
AD  - Department of Rheumatology, South Campus, Ren Ji Hospital, School of Medicine, 
      Shanghai Jiaotong University, Shanghai, China.
FAU - Wang, Xiao-Dong
AU  - Wang XD
AD  - Department of Rheumatology, South Campus, Ren Ji Hospital, School of Medicine, 
      Shanghai Jiaotong University, Shanghai, China.
FAU - Wu, Chun-Yan
AU  - Wu CY
AD  - Department of Pathology, Shanghai Pulmonary Hospital, Tongji University, School 
      of Medicine, Shanghai, China.
FAU - Huang, Yan
AU  - Huang Y
AD  - Department of Pathology, Shanghai Pulmonary Hospital, Tongji University, School 
      of Medicine, Shanghai, China.
FAU - Zhang, Ai-Hong
AU  - Zhang AH
AD  - Department of Medical Statistics, Tongji University, School of Medicine, 
      Shanghai, China.
FAU - Li, Hui-Ping
AU  - Li HP
AD  - Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji 
      University, School of Medicine, Shanghai, China. liw2013@126.com.
LA  - eng
PT  - Journal Article
DEP - 20210330
PL  - Italy
TA  - Clin Exp Rheumatol
JT  - Clinical and experimental rheumatology
JID - 8308521
RN  - 0 (Pyridones)
RN  - D7NLD2JX7U (pirfenidone)
SB  - IM
MH  - Humans
MH  - *Idiopathic Pulmonary Fibrosis/drug therapy
MH  - *Lung Diseases, Interstitial/diagnosis
MH  - Pyridones/adverse effects
MH  - Retrospective Studies
MH  - Vital Capacity
EDAT- 2021/04/07 06:00
MHDA- 2022/04/01 06:00
CRDT- 2021/04/06 17:16
PHST- 2021/01/31 00:00 [received]
PHST- 2021/03/15 00:00 [accepted]
PHST- 2021/04/07 06:00 [pubmed]
PHST- 2022/04/01 06:00 [medline]
PHST- 2021/04/06 17:16 [entrez]
AID - 17028 [pii]
AID - 10.55563/clinexprheumatol/off5n7 [doi]
PST - ppublish
SO  - Clin Exp Rheumatol. 2022 Mar;40(3):560-567. doi: 
      10.55563/clinexprheumatol/off5n7. Epub 2021 Mar 30.