Department of Rheumatology, Ome Municipal General Hospital, Ome, Japan.
Department of Rheumatology and Medicine, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.
Mod Rheumatol. 2022 Aug 20;32(5):915-922. doi: 10.1093/mr/roab088.
In Japan, clinical records of patients with intractable diseases, including microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA), are compiled into a database. This study aimed to understand the current treatment status and changes in treatment regimens from our previous survey.
Using data from 2012 and 2013, patients with new-onset MPA and GPA were extracted and analysed.
We analysed 1278 MPA and 215 GPA patients. The average age was 71.7 and 62.7 years, respectively. Methylprednisolone pulse therapy was used in 51.2% of MPA patients and 40.5% of GPA patients; the initial prednisolone-equivalent glucocorticoid dose was 39.5 mg/day in MPA and 46.6 mg/day in GPA. Concomitant intravenous or oral cyclophosphamide (CY) was administered to 22.6% of MPA and 56.3% of GPA. Young age, bloody sputum, low serum creatinine, and high C-reactive protein levels were independently associated with CY use in MPA. Compliance with treatment protocol for Japanese patients with myeloperoxidase (MPO)-anti-neutrophilic cytoplasmic antibody-associated vasculitis study criteria or the 2011 clinical practice guidelines for rapidly progressive glomerulonephritis was 42.7% and 49.7%, respectively.
MPA was more prevalent than GPA in the registry. Compared to patients with GPA, MPA patients were older and used CY less frequently. No apparent changes in treatment trends were observed from the previous survey.
在日本，将包括显微镜下多血管炎（MPA）和肉芽肿性多血管炎（GPA）在内的难治性疾病患者的临床记录编制成数据库。本研究旨在了解与我们之前的调查相比，目前的治疗状况和治疗方案的变化。
使用 2012 年和 2013 年的数据，提取和分析新诊断的 MPA 和 GPA 患者的数据。
我们分析了 1278 例 MPA 和 215 例 GPA 患者。平均年龄分别为 71.7 岁和 62.7 岁。51.2%的 MPA 患者和 40.5%的 GPA 患者接受了甲泼尼龙脉冲治疗；MPA 患者的初始泼尼松等效糖皮质激素剂量为 39.5mg/天，GPA 患者为 46.6mg/天。22.6%的 MPA 和 56.3%的 GPA 患者接受了静脉或口服环磷酰胺（CY）治疗。年轻、血性痰、低血清肌酐和高 C 反应蛋白水平与 MPA 患者使用 CY 独立相关。符合日本抗髓过氧化物酶（MPO）-抗中性粒细胞胞质抗体相关性血管炎研究标准或 2011 年快速进展性肾小球肾炎临床实践指南的治疗方案的患者比例分别为 42.7%和 49.7%。
该登记处中 MPA 的发病率高于 GPA。与 GPA 患者相比，MPA 患者年龄更大，使用 CY 的频率更低。与之前的调查相比，治疗趋势没有明显变化。
