Ahmad Rawaha, Nahar Noor Un, Kiani Ismaa, Imran Muqaddas, Sultan Muhammad Umair
Acute Medicine, Northampton General Hospital, Northampton, GBR.
Nephrology, Northampton General Hospital, Northampton, GBR.
Cureus. 2025 Aug 14;17(8):e90094. doi: 10.7759/cureus.90094. eCollection 2025 Aug.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) includes a group of autoimmune disorders resulting in the production of autoantibodies to neutrophil proteins (leukocyte proteins, proteinase 3 (PR3)-ANCA or myeloperoxidase proteins (MPO)-ANCA). There are three subtypes of AAV based on ANCA serotypes: PR3+ AAV, MPO+ AAV, and ANCA+. These rare disorders present with a wide spectrum of symptoms with involvement of multiple organs, including the heart, kidneys, eyes, ears, brain, spinal cord, nerves, and musculoskeletal system, making them difficult to diagnose promptly. This impacts the prognosis of AAV, as involvement of major organs can increase recurrence rates and lead to a poor prognosis, while timely diagnosis can lead to full recovery. Treatment options mainly involve immunosuppression with steroids, rituximab, methotrexate, and cyclophosphamide to maintain remission. We discuss a case of granulomatosis with polyangiitis in an adult male. We believe this report will help highlight timely diagnosis and interventions, overall improving the prognosis of patients.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）包括一组自身免疫性疾病，可导致产生针对中性粒细胞蛋白（白细胞蛋白、蛋白酶3（PR3）-ANCA或髓过氧化物酶蛋白（MPO）-ANCA）的自身抗体。根据ANCA血清型，AAV有三种亚型：PR3+ AAV、MPO+ AAV和ANCA+。这些罕见疾病表现出广泛的症状，累及多个器官，包括心脏、肾脏、眼睛、耳朵、大脑、脊髓、神经和肌肉骨骼系统，因此难以迅速诊断。这会影响AAV的预后，因为主要器官受累会增加复发率并导致预后不良，而及时诊断则可实现完全康复。治疗选择主要包括使用类固醇、利妥昔单抗、甲氨蝶呤和环磷酰胺进行免疫抑制以维持缓解。我们讨论了一例成年男性肉芽肿性多血管炎病例。我们相信本报告将有助于突出及时诊断和干预，总体上改善患者的预后。
