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抗中性粒细胞胞浆抗体相关血管炎患者死亡率的肺部和肾脏预测因素:来自土耳其的地区经验。

Pulmonary and Renal Predictors of Mortality in ANCA-Associated Vasculitis: A Regional Experience from Türkiye.

作者信息

Bulut Gökten Dilara, Karabağ Sevil, Mercan Rıdvan

机构信息

Department of Rheumatology, Tekirdag Namik Kemal University, Tekirdag 59059, Türkiye.

Department of Pathology, Tekirdag Namik Kemal University, Tekirdag 59059, Türkiye.

出版信息

Biomedicines. 2025 Jun 7;13(6):1401. doi: 10.3390/biomedicines13061401.

Abstract

: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare autoimmune disease marked by small-vessel inflammation. Pulmonary and renal manifestations are believed to critically influence prognosis, but detailed regional data are lacking. This study aimed to determine the prevalence and prognostic impact of pulmonary and renal involvement in AAV patients in the Thrace region of Türkiye. : A retrospective cohort study was conducted on 78 biopsy-proven AAV patients followed between 2018 and 2025. Demographic, clinical, laboratory, and outcome data were analysed. Logistic regression identified predictors of relapse and mortality. : The cohort included 44 granulomatosis with polyangiitis, 30 microscopic polyangiitis, and 4 eosinophilic granulomatosis with polyangiitis patients; 40 were pr3-ANCA positive and 33 MPO-ANCA positive. Pulmonary involvement was observed in 71.8% and renal involvement in 74.4%, and overall mortality was 20.5%. All deaths occurred in patients with pulmonary involvement (28.6% vs. 0%, = 0.048). Relapse was higher in those with pulmonary (17.9% vs. 4.5%, = 0.048) and renal (15.5% vs. 5%, = 0.056) involvement. Multivariate analysis showed that pulmonary involvement (OR 3.82, = 0.002), renal involvement (OR 4.73, = 0.013), and rituximab treatment (OR 10.79, = 0.049) predicted relapse; elevated CRP (OR 1.01, = 0.003), creatinine (OR 1.42, = 0.028), hypoalbuminaemia (OR 0.24, = 0.046), renal (OR 2.86, = 0.031), and pulmonary (OR 3.21, = 0.003) involvement predicted mortality. : Pulmonary and renal involvement are highly prevalent and represent the strongest predictors of relapse and mortality in AAV patients in this regional cohort. Recognising these risks is essential to guide early interventions and improve patient outcomes.

摘要

抗中性粒细胞胞浆抗体(ANCA)相关血管炎是一种以小血管炎症为特征的罕见自身免疫性疾病。肺部和肾脏表现被认为对预后有至关重要的影响,但缺乏详细的区域数据。本研究旨在确定土耳其色雷斯地区AAV患者肺部和肾脏受累的患病率及其对预后的影响。

对2018年至2025年期间随访的78例经活检证实的AAV患者进行了一项回顾性队列研究。分析了人口统计学、临床、实验室和结局数据。逻辑回归确定了复发和死亡的预测因素。

该队列包括44例肉芽肿性多血管炎、30例显微镜下多血管炎和4例嗜酸性肉芽肿性多血管炎患者;40例为pr3-ANCA阳性,33例为MPO-ANCA阳性。观察到肺部受累的比例为71.8%,肾脏受累的比例为74.4%,总死亡率为20.5%。所有死亡均发生在肺部受累的患者中(28.6%对0%,P = 0.048)。肺部受累(17.9%对4.5%,P = 0.048)和肾脏受累(15.5%对5%,P = 0.056)的患者复发率更高。多变量分析显示,肺部受累(比值比3.82,P = 0.002)、肾脏受累(比值比4.73,P = 0.013)和利妥昔单抗治疗(比值比10.79,P = 0.049)可预测复发;CRP升高(比值比1.01,P = 0.003)、肌酐(比值比1.42,P = 0.028)、低白蛋白血症(比值比0.24,P = 0.046)、肾脏受累(比值比2.86,P = 0.031)和肺部受累(比值比3.21,P = 0.003)可预测死亡。

肺部和肾脏受累非常普遍,是该地区队列中AAV患者复发和死亡的最强预测因素。认识到这些风险对于指导早期干预和改善患者结局至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ade5/12189655/cfff2b9f3a8a/biomedicines-13-01401-g001.jpg

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