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在一家哥伦比亚医院中,抗中性粒细胞胞浆抗体相关性血管炎患者的临床特征和结局。

Clinical Characteristics and Outcomes of Patients With ANCA-Associated Vasculitides in a Colombian Hospital.

机构信息

From the Medical School, Universidad Icesi.

Centro de Investigaciones Clínicas, Fundación Valle del Lili.

出版信息

J Clin Rheumatol. 2022 Mar 1;28(2):e491-e497. doi: 10.1097/RHU.0000000000001775.

Abstract

BACKGROUND/OBJECTIVE: Antineutrophil cytoplasmic antibody-associated vasculitides (AAVs) are uncommon systemic autoimmune diseases, of which few reports exist in Latin America. Our aim was to examine AAV evaluated in a high-complexity hospital in southwestern Colombia, with emphasis in severe forms.

METHODS

A medical records review study of 67 patients was performed, and data were collected from electronic registries. Moderate and severe AAVs were defined as the presence of life-threatening complications, unfavorable Birmingham Vasculitis Activity Score outcomes, and hospitalization requirements at the time of diagnosis and by the last follow-up, between 2011 and 2019. Clinical manifestations, treatment, and outcomes were evaluated. The AAV subtypes were compared.

RESULTS

A total of 67 cases were included. The majority were female (n = 44, 65.67%), and the median age was 52 (40-64) years. Granulomatosis with polyangiitis (GPA) was the most frequent with 42 patients (62.68%), followed by microscopic polyangiitis (MPA) and eosinophilic GPA, with 15 patients (22.38%) and 10 patients (14.92%), respectively. Forty-four patients (65.67%) presented pulmonary symptoms. The highest Birmingham Vasculitis Activity Score corresponded to MPA, with 21 (12-25) points. Fifteen patients (22.4%) were admitted to the intensive care unit throughout the course of the disease, of whom 10 had GPA. The longest stay and duration of mechanical ventilation were seen in MPA. The principal treatments were corticosteroids and cyclophosphamide, and the main outcome was end-stage renal disease.

CONCLUSIONS

In this cohort of AAV, most of cases corresponded to GPA, and pulmonary manifestations were the most common. Microscopic polyangiitis was the more severe subtype as it showed worse impairment in clinical characteristics and intensive care unit requirements.

摘要

背景/目的:抗中性粒细胞胞浆抗体相关性血管炎(AAV)是一种罕见的系统性自身免疫性疾病,在拉丁美洲的报道较少。我们的目的是研究在哥伦比亚西南部一家高复杂性医院评估的 AAV,并重点关注严重形式。

方法

对 2011 年至 2019 年间 67 例患者的病历进行回顾性研究,数据来自电子登记册。中度和重度 AAV 的定义为存在危及生命的并发症、不良的伯明翰血管炎活动评分结果以及诊断时和最后一次随访时需要住院治疗。评估了临床表现、治疗和结局。比较了 AAV 亚型。

结果

共纳入 67 例病例。大多数为女性(n = 44,65.67%),中位年龄为 52(40-64)岁。肉芽肿性多血管炎(GPA)最常见,有 42 例(62.68%),其次是显微镜下多血管炎(MPA)和嗜酸性粒细胞性 GPA,分别有 15 例(22.38%)和 10 例(14.92%)。44 例(65.67%)患者有肺部症状。最高的伯明翰血管炎活动评分属于 MPA,为 21(12-25)分。15 例(22.4%)患者在疾病过程中被收入重症监护病房,其中 10 例患有 GPA。MPA 患者的住院时间最长,需要机械通气的时间最长。主要治疗方法是皮质类固醇和环磷酰胺,主要结局是终末期肾病。

结论

在该 AAV 队列中,大多数病例属于 GPA,肺部表现最为常见。显微镜下多血管炎是更严重的亚型,因为它在临床特征和重症监护病房需求方面表现出更严重的损害。

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