Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Division of Pathology, Gifu University Hospital, Gifu, Japan.
Mod Rheumatol Case Rep. 2020 Jan;4(1):63-69. doi: 10.1080/24725625.2019.1673528. Epub 2019 Oct 23.
Immunoglobulin A (IgA) vasculitis (IgAV) is a small vessel vasculitis presenting cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis caused by deposition of the IgA-mediated immune complex. Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by eosinophil-rich and granulomatous inflammation in small to medium-sized vessels. Both IgAV and EGPA are classified as autoimmune systemic vasculitis, but the pathogenesis of immune complex-mediated IgAV and that of pauci-immune EGPA are different. Here we report a rare case of simultaneous development of IgAV and EGPA presenting palpable purpura and numbness in a patient with a history of asthma. Histological examination revealed leukocytoclastic vasculitis with deposition of IgA, IgM and C3 in the upper dermis and necrotising vasculitis with eosinophilic infiltration and granulomatous formation in the lower dermis and subcutaneous fat, indicating the existence of IgAV and EGPA. Our case provides evidence of concurrent development of two different types of vasculitis, which may affect disease-associated complications, therapeutic strategy and prognosis.
免疫球蛋白 A (IgA) 血管炎 (IgAV) 是一种小血管血管炎，表现为皮肤紫癜、关节痛和/或关节炎、急性肠炎和由 IgA 介导的免疫复合物沉积引起的肾小球肾炎。嗜酸性肉芽肿性多血管炎 (EGPA) 是一种抗中性粒细胞胞质抗体 (ANCA) 相关性血管炎 (AAV)，其特征是小到中等大小血管中富含嗜酸性粒细胞和肉芽肿性炎症。IgAV 和 EGPA 均被归类为自身免疫性系统性血管炎，但免疫复合物介导的 IgAV 的发病机制和寡免疫性 EGPA 的发病机制不同。在这里，我们报告了一例罕见的同时发生的 IgAV 和 EGPA 的病例，表现为哮喘病史患者出现可触及的紫癜和麻木。组织学检查显示在上皮中存在白细胞碎裂性血管炎，伴有 IgA、IgM 和 C3 的沉积，在下皮和皮下脂肪中存在伴有嗜酸性粒细胞浸润和肉芽肿形成的坏死性血管炎，提示存在 IgAV 和 EGPA。我们的病例提供了两种不同类型血管炎同时发生的证据，这可能会影响与疾病相关的并发症、治疗策略和预后。
