Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature.
Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature.
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DOI：
10.1097/md.0000000000000217
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发表时间：
2014-11
期刊：
影响因子：
1.6
通讯作者：
Saadoun D
中科院分区：
文献类型：
--
作者：
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
肺纤维化（PF）是一种罕见的表现，观察到患者与抗神经细胞胞浆抗体（ANCA）相关的血管炎（AAV），特别是显微镜下多血管炎（MPA）。虽然与AAV相关的PF患者似乎预后较差，但这些患者仅在病例报告或小型回顾性病例系列中描述。在这项回顾性多中心研究中，我们报告了符合美国流变学学会标准和/或查佩尔山定义的与AAV相关的PF患者的主要特征和长期结局。确定了49例与AAV相关的PF患者（30例男性[61%];诊断AAV时的中位年龄为68 [四分位数间距，58-73]岁）。40例（81.6%）患者患有MPA，9例（18.4%）患者患有肉芽肿性多血管炎。在22例（45%）患者中，PF的诊断先于血管炎发作。肺间质性肺炎是主要的影像学类型（n = 18，43%）。 ANCA多为抗髓过氧化物酶特异性（88%）。所有患者均接受糖皮质激素诱导治疗，联合环磷酰胺（CYC）（n = 36，73.5%）或利妥昔单抗（RTX）（n = 1，2%）。 与死亡率相关的因素包括慢性呼吸功能不全的发生（风险比[HR]，7.44; 95%置信区间[CI]，1.6-34.5; p = 0.003）、糖皮质激素单独诱导治疗（HR，2.94; CI，1.05-8.33; p = 0.04）和初始体重减轻（HR，2.83; CI，1.05-7.65; p = 0.041）。 单独使用糖皮质激素或联合免疫抑制剂（CYC或RTX）作为诱导治疗的患者的3年生存率分别为64%（95%CI，41-99）和94%（95%CI，86-100）（p = 0.03）。 中位随访48个月后[四分位距，14-88个月]，18例（37%）患者死亡，包括11例与呼吸功能不全相关。PF是AAV的一种罕见表现，预后非常差。CYC诱导治疗可改善预后。
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.