抗中性粒细胞胞浆抗体相关血管炎疾病表现的深入分析确定了不同的临床表型。
In-Depth Analysis of Disease Manifestations in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides Identifies Distinct Clinical Phenotypes.
作者信息
Lindberg Hanna, Knight Ann, Hellbacher Erik, Norling Olof, Berglin Ewa, Stegmayr Bernd, Baslund Bo, Palm Øyvind, Haukeland Hilde, Gunnarsson Iva, Bruchfeld Annette, Weiner Maria, Eriksson Per, Segelmark Mårten, Ohlsson Sophie, Mohammad Aladdin J, Svärd Anna, Pullerits Rille, Herlitz Hans, Söderbergh Annika, Rantapää-Dahlqvist Solbritt, Dahlqvist Johanna
机构信息
Uppsala University and Uppsala University Hospital, Uppsala, Sweden.
Uppsala University, Uppsala, Sweden.
出版信息
ACR Open Rheumatol. 2025 Mar;7(3):e70009. doi: 10.1002/acr2.70009.
OBJECTIVE
The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are heterogeneous disorders. The aim of this study was to identify and characterize subgroups of patients based on sex, ANCA, age at diagnosis, and organ involvement.
METHODS
In total, 1,167 patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) were retrospectively recruited to the study. Data including cumulative involvement of 10 different organ systems, end-stage kidney disease (ESKD), sex, proteinase (PR) 3-ANCA, myeloperoxidase (MPO)-ANCA, age at diagnosis, disease duration, and relapse were obtained from medical records. Clinical variables were analyzed for associations with sex, age at diagnosis, and relapse using logistic regression analysis. Thirteen clinical variables were included in hierarchical cluster analyses using the Ward method.
RESULTS
In patients with GPA, PR3-ANCA, renal and pulmonary involvement, and ESKD were significantly associated with male sex, whereas MPO-ANCA was associated with female sex. Patients with GPA who were younger than 32 years of age at diagnosis were significantly more often females and had more ear-nose-throat involvement than patients older than 32 years. In patients with MPA, female patients were significantly younger at diagnosis than male patients. Relapse was significantly associated with young age at diagnosis and pulmonary involvement in GPA and with musculoskeletal involvement in MPA. Hierarchical cluster analyses identified five and seven patient clusters among individuals with GPA and MPA, respectively. PR3-/MPO-ANCA defined the largest clusters, whereas heart, gastrointestinal, and central nervous system involvement were hallmarks for three clusters for both patients with GPA and MPA.
CONCLUSION
Sex, age at diagnosis, and specific organ involvements define clinically relevant subgroups among patients with ANCA-associated vasculitides.
目的
抗中性粒细胞胞浆抗体（ANCA）相关血管炎是一组异质性疾病。本研究的目的是根据性别、ANCA、诊断时年龄和器官受累情况来识别和描述患者亚组。
方法
本研究共回顾性纳入了1167例肉芽肿性多血管炎（GPA）或显微镜下多血管炎（MPA）患者。从病历中获取包括10个不同器官系统的累积受累情况、终末期肾病（ESKD）、性别、蛋白酶（PR）3-ANCA、髓过氧化物酶（MPO）-ANCA、诊断时年龄、疾病持续时间和复发情况等数据。使用逻辑回归分析临床变量与性别、诊断时年龄和复发的相关性。采用Ward法将13个临床变量纳入分层聚类分析。
结果
在GPA患者中，PR3-ANCA、肾脏和肺部受累以及ESKD与男性显著相关，而MPO-ANCA与女性相关。诊断时年龄小于32岁的GPA患者女性比例显著更高，且耳鼻喉受累情况比32岁以上患者更多。在MPA患者中，女性诊断时年龄显著小于男性。复发与GPA诊断时年轻及肺部受累以及MPA的肌肉骨骼受累显著相关。分层聚类分析分别在GPA和MPA患者中识别出5个和7个患者聚类。PR3-/MPO-ANCA定义了最大的聚类，而心脏、胃肠道和中枢神经系统受累是GPA和MPA患者三个聚类的特征。
结论
性别、诊断时年龄和特定器官受累情况定义了ANCA相关血管炎患者中具有临床意义的亚组。
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