Department of Clinical Immunology and Rheumatology, Mahatma Gandhi Medical College and Hospital, Jaipur, India.
Clinical Immunology and Rheumatology Division, Department of Internal Medicine, PGIMER, Chandigarh, India.
Autoimmun Rev. 2024 Nov;23(11):103647. doi: 10.1016/j.autrev.2024.103647. Epub 2024 Sep 28.
The ACR in 2021 and the EULAR in 2022 published recommendations for management of ANCA-associated vasculitis. Given the differences in the demographic, clinical profiles, and the socio-economic realities between various countries, there is a need for development of guidelines for the management of AAV for less economically developed regions of the world.
These guidelines were made following the GRADE methodology. After the systematic literature review, recommendations were formulated and opinion was sought from the 18-member expert panel consisting of 17 clinicians and one patient representative.
Twenty recommendations were formulated. We recommend ANCA testing by ELISA over IIF. For remission induction in active GPA or MPA, we recommend use of intravenous cyclophosphamide or rituximab in combination with glucocorticoids. We conditionally recommend the use of reduced dose glucocorticoids over standard dose glucocorticoids for remission induction in active GPA or MPA. For remission maintenance in patients with GPA or MPA, we recommend the use of rituximab over azathioprine for at least 48 months from diagnosis. We conditionally recommend the use of plasma exchange in patients with severe renal vasculitis. For remission induction in EGPA, we recommend use of cyclophosphamide or rituximab in severe disease and mepolizumab or azathioprine or methotrexate or mycophenolate mofetil in non-severe disease.
These are the first ever Indian recommendations for the management of AAV. Despite our effort to formulate these recommendations based on high quality evidence, some recommendations were still based on low quality evidence but with high rate of agreement among expert panel members.
2021 年美国风湿病学会（ACR）和 2022 年欧洲抗风湿病联盟（EULAR）发布了抗中性粒细胞胞浆抗体（ANCA）相关性血管炎的管理建议。鉴于各国在人口统计学、临床特征和社会经济现实方面存在差异，因此需要为世界上欠发达地区制定管理抗中性粒细胞胞浆抗体相关性血管炎的指南。
这些指南是按照 GRADE 方法制定的。在系统文献回顾后，制定了建议，并向由 17 名临床医生和 1 名患者代表组成的 18 人专家小组征求意见。
制定了 20 条建议。我们建议通过酶联免疫吸附试验（ELISA）而非间接免疫荧光法（IIF）进行 ANCA 检测。对于活动型 GPA 或 MPA 的缓解诱导，我们建议使用静脉注射环磷酰胺或利妥昔单抗联合糖皮质激素。我们有条件地建议在活动型 GPA 或 MPA 的缓解诱导中使用低剂量糖皮质激素替代标准剂量糖皮质激素。对于 GPA 或 MPA 患者的缓解维持，我们建议从诊断开始至少使用利妥昔单抗 48 个月。我们有条件地建议在严重肾血管炎患者中使用血浆置换。对于 EGPA 的缓解诱导，我们建议在严重疾病中使用环磷酰胺或利妥昔单抗，在非严重疾病中使用美泊利珠单抗、吗替麦考酚酯、阿扎胞苷或甲氨蝶呤。
这是印度首次制定的抗中性粒细胞胞浆抗体相关性血管炎管理建议。尽管我们努力根据高质量证据制定这些建议，但有些建议仍然基于低质量证据，但专家小组成员的共识率很高。
