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10.1038/srep33226

http://scihub22266oqcxt.onion/10.1038/srep33226
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suck abstract from ncbi


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pmid27615411
      Sci+Rep 2016 ; 6 (ä): 33226
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  • Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis #MMPMID27615411
  • Li T ; Guo L ; Chen Z ; Gu L ; Sun F ; Tan X ; Chen S ; Wang X ; Ye S
  • Sci Rep 2016[Sep]; 6 (ä): 33226 PMID27615411 show ga
  • 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.
  • |Adult [MESH]
  • |Anti-Inflammatory Agents, Non-Steroidal/*therapeutic use [MESH]
  • |Dermatomyositis/complications/*drug therapy/mortality [MESH]
  • |Disease Progression [MESH]
  • |Female [MESH]
  • |Humans [MESH]
  • |Kaplan-Meier Estimate [MESH]
  • |Lung Diseases, Interstitial/*drug therapy/etiology/mortality/pathology [MESH]
  • |Male [MESH]
  • |Middle Aged [MESH]
  • |Prospective Studies [MESH]
  • |Pyridones/*therapeutic use [MESH]
  • |Retrospective Studies [MESH]


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