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肉芽肿性多血管炎和显微镜下多血管炎的治疗方法。

Treatment Approaches to Granulomatosis with Polyangiitis and Microscopic Polyangiitis.

机构信息

Center for Medical Sciences (CISMed), Department of Cellular, Computational and Integrative Biology (CIBIO), University of Trento, and Division of Rheumatology, Santa Chiara Hospital, APSS Trento, Italy.

Rheumatology Department, INSERM UMR1227 LBAI, Lymphocytes B, Autoimmunité et Immunothérapies, University of Brest, National Reference Center for Rare Systemic Autoimmune Diseases CERAINO, CHRU Brest, Brest, France.

出版信息

Rheum Dis Clin North Am. 2023 Aug;49(3):545-561. doi: 10.1016/j.rdc.2023.03.004. Epub 2023 Apr 28.

Abstract

We have made significant headway in our ability to induce and maintain remission in patients with granulomatosis with polyangiitis and microscopic polyangiitis. With increased understanding of the pathogenesis of antineutrophilic cytoplasmic antibody-associated vasculitides (AAV), therapeutic targets have been identified and studied in clinical trials. From initial induction strategies including glucocorticoids and cyclophosphamide, we have discovered effective induction regimens with rituximab and complement inhibition that can significantly decrease the glucocorticoid cumulative doses in patients with AAV. There are many trials underway evaluating management strategies for refractory patients and exploring new and old therapies that may help to continuously improve outcomes for patients with AAV.

摘要

我们在诱导和维持肉芽肿性多血管炎和显微镜下多血管炎患者缓解方面取得了重大进展。随着对抗中性粒细胞胞浆抗体相关性血管炎(AAV)发病机制的深入了解,已确定并在临床试验中研究了治疗靶点。从最初包括糖皮质激素和环磷酰胺在内的诱导策略,我们发现了有效的诱导方案,包括利妥昔单抗和补体抑制,这可以显著减少 AAV 患者的糖皮质激素累积剂量。目前有许多临床试验正在评估难治性患者的治疗策略,并探索新的和旧的治疗方法,以帮助不断改善 AAV 患者的预后。

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