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日本日常临床实践中晚发型肉芽肿性多血管炎/显微镜下多血管炎患者与早发型患者的结局比较:日本一项两中心回顾性研究

Comparison of Outcomes Between Japanese Patients With Older-Onset Granulomatosis With Polyangiitis/Microscopic Polyangiitis and Younger-Onset Patients in Daily Clinical Practice: A Two-Center Retrospective Study in Japan.

作者信息

Sato Shuzo, Yashiro-Furuya Makiko, Sumichika Yuya, Saito Kenji, Yoshida Shuhei, Matsumoto Haruki, Temmoku Jumpei, Fujita Yuya, Matsuoka Naoki, Asano Tomoyuki, Suzuki Eiji, Kanno Takashi, Migita Kiyoshi

机构信息

Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan.

Department of Rheumatology, Ohta-Nishinouchi Hospital, Fukushima, Japan.

出版信息

Int J Rheum Dis. 2025 May;28(5):e70256. doi: 10.1111/1756-185X.70256.

DOI:10.1111/1756-185X.70256
PMID:40329657
Abstract

AIMS

Although previous studies have reported poor outcomes in older-onset (≥ 75 years old) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) compared with younger-onset AAV, the distinct cause of poor prognosis remains unclear. This study aimed to investigate the clinical features, therapies, and outcomes of older patients with granulomatosis with polyangiitis (GPA) and patients with microscopic polyangiitis (MPA) compared to younger-onset patients.

METHODS

This two-center retrospective cohort study enrolled 70 newly-onset Japanese patients with AAV (GPA and MPA) from the Fukushima Medical University Hospital and Ohta-Nishinouchi Hospital in Fukushima, Japan, between 2004 and 2019. Clinical records were retrospectively reviewed, and clinical features and outcomes (1-year and 3-year survival by the Kaplan-Meier method) were compared between older and younger GPA/MPA groups, respectively.

RESULTS

Clinical features of the older GPA/MPA group were similar to those of the younger GPA/MPA group; however, the older GPA group showed severe inflammation and the older MPA group had an increased frequency of renal involvement and fever. The 1-year survival in the older MPA group was significantly lower than that in the younger MPA group. Immunosuppressive therapy including cyclophosphamide, rituximab, and other immunosuppressive agents was important to sustain the survival of patients with GPA/MPA.

CONCLUSIONS

Older patients with GPA/MPA may have specific clinical features; careful observation is needed during the treatment of older patients with MPA. Immunosuppressive therapy may improve the prognosis of patients with AAV.

摘要

目的

尽管先前的研究报告称,与早发型抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)相比,晚发型(≥75岁)AAV的预后较差,但预后不良的具体原因仍不清楚。本研究旨在调查与早发型患者相比,老年肉芽肿性多血管炎(GPA)患者和显微镜下多血管炎(MPA)患者的临床特征、治疗方法及预后情况。

方法

这项双中心回顾性队列研究纳入了2004年至2019年间来自日本福岛医科大学医院和大田西之内医院的70例新诊断为AAV(GPA和MPA)的日本患者。对临床记录进行回顾性分析,分别比较老年和年轻GPA/MPA组的临床特征和预后(采用Kaplan-Meier法计算1年和3年生存率)。

结果

老年GPA/MPA组的临床特征与年轻GPA/MPA组相似;然而,老年GPA组炎症更为严重,老年MPA组肾脏受累和发热的频率更高。老年MPA组的1年生存率显著低于年轻MPA组。包括环磷酰胺、利妥昔单抗和其他免疫抑制剂在内的免疫抑制治疗对于维持GPA/MPA患者的生存至关重要。

结论

老年GPA/MPA患者可能具有特定的临床特征;在治疗老年MPA患者时需要仔细观察。免疫抑制治疗可能改善AAV患者的预后。

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