Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, Mexico City, Mexico, 14080.
Department of Nephrology and Mineral Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Immunol Res. 2022 Aug;70(4):550-559. doi: 10.1007/s12026-022-09279-8. Epub 2022 Apr 21.
Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides are infrequent autoimmune diseases characterized by inflammation of the walls of small vessels leading to tissue and endothelial damage. On the other hand, IgG4-related disease is a fibroinflammatory disease characterized histologically by lymphoplasmacytic infiltrates with IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis that may affect nearly every organ of the body. There are similarities in clinical, serological, radiological, and histopathological features between both diseases, and hence, they usually mimic each other complicating the differential diagnosis. Furthermore, reports of patients with the coexistence of both conditions (overlap syndrome) have been reported. We herein report a patient with an unequivocal diagnosis of ANCA-associated vasculitis, specifically granulomatosis with polyangiitis (posterior uveitis, polyneuropathy, pauci-immune glomerulonephritis with crescent formation and granulomas, and MPO-ANCA positivity) and IgG4-related disease (thoracic aortitis, tubulointerstitial nephritis with prominent IgG4+ plasma cell infiltration, fibrosis, and obliterative arteritis, high levels of serum IgG4, and eosinophilia) overlap syndrome.
抗中性粒细胞胞质抗体（ANCA）相关性血管炎是一种罕见的自身免疫性疾病，其特征为小血管壁炎症，导致组织和内皮损伤。另一方面，IgG4 相关疾病是一种纤维炎症性疾病，组织学上表现为淋巴浆细胞浸润伴 IgG4+浆细胞、席纹状纤维化和闭塞性静脉炎，可能影响身体的几乎每个器官。这两种疾病在临床、血清学、影像学和组织病理学特征上存在相似之处，因此常相互模仿，导致鉴别诊断困难。此外，还报道了同时存在这两种疾病（重叠综合征）的患者。本文报告了一例明确诊断为 ANCA 相关性血管炎的患者，具体为肉芽肿性多血管炎（后葡萄膜炎、多发性神经病、少免疫性新月体性肾小球肾炎伴新月体形成和肉芽肿、MPO-ANCA 阳性）和 IgG4 相关疾病（胸主动脉炎、伴有明显 IgG4+浆细胞浸润、纤维化和闭塞性动脉炎的肾小管间质性肾炎、高血清 IgG4 水平和嗜酸性粒细胞增多）重叠综合征。
