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2014 ; 93
(24
): 340-349
Nephropedia Template TP
gab.com Text
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English Wikipedia
Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated
vasculitis: a series of 49 patients and review of the literature
#MMPMID25500703
Comarmond C
; Crestani B
; Tazi A
; Hervier B
; Adam-Marchand S
; Nunes H
; Cohen-Aubart F
; Wislez M
; Cadranel J
; Housset B
; Lloret-Linares C
; Sève P
; Pagnoux C
; Abad S
; Camuset J
; Bienvenu B
; Duruisseaux M
; Hachulla E
; Arlet JB
; Hamidou M
; Mahr A
; Resche-Rigon M
; Brun AL
; Grenier P
; Cacoub P
; Saadoun D
Medicine (Baltimore)
2014[Nov]; 93
(24
): 340-349
PMID25500703
show ga
Pulmonary fibrosis (PF) is an uncommon manifestation observed in patients with
antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV),
particularly microscopic polyangiitis (MPA). While patients with PF associated
with AAV seem to have a worse prognosis, these patients have been described only
in case reports or small retrospective case series. In this retrospective
multicenter study, we report the main features and long-term outcomes of patients
with PF associated with AAV, fulfilling the American College of Rheumatology
criteria and/or Chapel Hill definitions. Forty-nine patients (30 men [61%];
median age at diagnosis of AAV, 68 [interquartile range, 58-73] years) with PF
associated with AAV were identified. Forty (81.6%) patients had MPA and 9 (18.4%)
had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of
vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main
radiologic pattern (n?=?18, 43%). ANCA were mostly of antimyeloperoxidase
specificity (88%). All patients were treated with glucocorticoids as induction
therapy, combined with cyclophosphamide (CYC) (n?=?36, 73.5%) or rituximab (RTX)
(n?=?1, 2%). Factors associated with mortality included occurrence of chronic
respiratory insufficiency (hazard ratio [HR], 7.44; 95% confidence interval [CI],
1.6-34.5; p?=?0.003), induction therapy with glucocorticoids alone (HR, 2.94; CI,
1.05-8.33; p?=?0.04), and initial weigh loss (HR, 2.83; CI, 1.05-7.65;
p?=?0.041). The 3-year survival rate in patients treated with glucocorticoids
alone or combined with an immunosuppressant (CYC or RTX) as induction therapy was
64% (95% CI, 41-99) and 94% (95% CI, 86-100), respectively (p?=?0.03). After a
median follow-up of 48 months [interquartile range, 14-88 mo], 18 (37%) patients
died, including 11 related to respiratory insufficiency. PF is a rare
manifestation of AAV with a very poor prognosis. Induction therapy with CYC might
improve the outcome.