Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Int J Rheum Dis. 2024 Jan;27(1):e14887. doi: 10.1111/1756-185X.14887. Epub 2023 Aug 23.
Rituximab (RTX) is being used for both induction and maintenance of anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis. However, the efficacy of RTX for the granulomatous findings of granulomatosis with polyangiitis (GPA) has not been demonstrated as clearly as its vasculitic manifestations.
A 46-year-old man was diagnosed in 2019 with GPA with constitutional symptoms, bilateral mastoiditis, prostatic necrosis, nodules in both lungs, pauci-immune necrotizing glomerulonephritis and high level of PR3-ANCA. He reached clinical remission after induction with high-dose corticosteroids and intravenous cyclophosphamide pulses at the 3rd month. Two months following the second cycle of RTX as maintenance, he developed multiple cranial mass lesions, and excisional biopsy revealed necrotizing vasculitis with granuloma formation. Remission was achieved with long-term high-dose corticosteroid therapy after surgical excision.
We observed a relapse of GPA with intracranial granulomatous lesions in a patient under RTX maintenance. Limited efficacy of RTX should be considered for mainly granulomatous manifestations in patients with GPA.
利妥昔单抗（RTX）既用于抗中性粒细胞胞浆抗体（ANCA）相关性血管炎的诱导缓解，也用于维持缓解。然而，RTX 对肉芽肿性多血管炎（GPA）的肉芽肿表现的疗效不如其血管炎表现那样明确。
一名 46 岁男性于 2019 年被诊断为 GPA，伴有全身症状、双侧乳突炎、前列腺坏死、双肺结节、少免疫性坏死性肾小球肾炎和 PR3-ANCA 水平升高。他在第 3 个月接受大剂量皮质类固醇和静脉环磷酰胺冲击治疗后达到临床缓解。RTX 维持治疗的第二个周期后 2 个月，他出现多个颅肿块病变，切除活检显示伴有肉芽肿形成的坏死性血管炎。在手术切除后，长期大剂量皮质类固醇治疗使他获得缓解。
我们观察到一名接受 RTX 维持治疗的患者出现 GPA 伴颅内肉芽肿性病变的复发。对于 GPA 患者主要表现为肉芽肿性病变时，RTX 的疗效可能有限。
