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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]