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肉芽肿性多血管炎发病率及复发的季节性变化:一项来自安纳托利亚中部的回顾性队列研究

Seasonal variation in incidence and relapse of granulomatosis with polyangiitis: A retrospective cohort study from Central Anatolia.

作者信息

Soytürk Gülşah, Konak Hatice Ecem, Dağlı Pınar Akyüz, Ulucaköy Rezan Koçak, Atalar Ebru, Erdoğan Esra Kayacan, Babaoğlu Hakan, Armağan Berkan, Orhan Kevser, Dogan İsmail, Maraş Yüksel, Omma Ahmet, Erten Şükran, Küçükşahin Orhan, Güven Serdar Can

机构信息

Ankara City Hospital, Rheumatology Clinic, Ankara, Turkey.

Yıldırım Beyazıt University, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey.

出版信息

Sarcoidosis Vasc Diffuse Lung Dis. 2025 Jun 25;42(2):16537. doi: 10.36141/svdld.v42i2.16537.

Abstract

BACKGROUND AND AIM

The etiology of granulomatosis with polyangiitis (GPA) remains under discussion. This study aims to explore patterns of organ involvement, ANCA antibody profiles, seasonal attack rates, and their interrelationship among patients diagnosed with ANCA-associated vasculitis (AAV) in Central Anatolia, shedding light on its multifactorial etiology involving drugs, genetics and environmental factors.

METHODS

We conducted a retrospective study involving patients aged 18 to 65 diagnosed with GPA and receiving care at Ankara Bilkent City Hospital Rheumatology Clinic. Diagnosis criteria followed the 2012 Chapel Hill Consensus Conference guidelines and the 2022 American College of Rheumatology/European Association of Rheumatology Societies classification for AAV. Patient data included demographics, antibody test results, seasons of diagnosis and flare, affected organs, and Birmingham Vasculitis Activity Score (BVAS). Organ involvement was determined based on biopsy findings or established criteria.

RESULTS

Our study included 75 patients, with the majority exhibiting cANCA-IFA positivity (94.7%) and PR3-ANCA ELISA positivity (98.3%). During follow-up, 70.7% experienced their first flare, with 37.7% experiencing a second flare. Lung involvement was most common at diagnosis and during flares, followed by ear-nose-throat and renal involvement. Seasonal analysis revealed peaks in disease onset in March, November, and April, with flares more common in September, May, October, and November. Autumn was the most common season for the first flare.

CONCLUSIONS

This study provides novel insights into ANCA-associated vasculitis epidemiology in Central Anatolia. Our findings underscore the intricate seasonal variation and infectious triggers of GPA exacerbations, highlighting the importance of tailored management strategies to mitigate disease flares.

摘要

背景与目的

肉芽肿性多血管炎(GPA)的病因仍在探讨中。本研究旨在探究安纳托利亚中部地区抗中性粒细胞胞浆抗体相关性血管炎(AAV)患者的器官受累模式、抗中性粒细胞胞浆抗体(ANCA)谱、季节性发作率及其相互关系,以阐明其涉及药物、遗传和环境因素的多因素病因。

方法

我们进行了一项回顾性研究,纳入了年龄在18至65岁之间、在安卡拉比尔肯特市医院风湿病诊所诊断为GPA并接受治疗的患者。诊断标准遵循2012年查珀尔希尔共识会议指南以及2022年美国风湿病学会/欧洲风湿病联盟关于AAV的分类标准。患者数据包括人口统计学信息、抗体检测结果、诊断和病情复发季节、受累器官以及伯明翰血管炎活动评分(BVAS)。根据活检结果或既定标准确定器官受累情况。

结果

我们的研究纳入了75例患者,大多数患者表现为胞浆型抗中性粒细胞胞浆抗体间接免疫荧光法(cANCA-IFA)阳性(94.7%)和蛋白酶3-抗中性粒细胞胞浆抗体酶联免疫吸附测定(PR3-ANCA ELISA)阳性(98.3%)。在随访期间,70.7%的患者经历了首次病情复发,37.7%的患者经历了第二次病情复发。肺部受累在诊断时和病情复发期间最为常见,其次是耳鼻喉和肾脏受累。季节性分析显示,疾病发病高峰出现在3月、11月和4月,病情复发在9月、5月、10月和11月更为常见。秋季是首次病情复发最常见的季节。

结论

本研究为安纳托利亚中部地区抗中性粒细胞胞浆抗体相关性血管炎的流行病学提供了新的见解。我们的研究结果强调了GPA病情加重的复杂季节性变化和感染诱因,突出了制定针对性管理策略以减轻疾病发作的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8987/12242877/1cfa7c5a38d8/SVDLD-42-16537-g001.jpg

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