医学
相伴的
抗中性粒细胞胞浆抗体
显微镜下多血管炎
IgG4相关疾病
免疫学
髓过氧化物酶
病理
肾小球肾炎
抗体
血管炎
纤维化
胃肠病学
内科学
肾
疾病
炎症
作者
Yan Ma,Liangliang Chen,Ying Xü,Qingpeng Han,Binfeng Yu,Yuan Yuan,Jie Zhao,Yi Yang,Jianghua Chen,Fei Han
标识
DOI:10.1111/1756-185x.13726
摘要
Abstract Aim The characteristics and the pathogenesis of the concomitant antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitides (AAV) and immunoglobulin G4‐related disease (IgG4‐RD) have not been elucidated. Method We included 92 AAV patients with renal biopsy results. Among them, 10 patients met both AAV and IgG4‐RD criteria (concomitant group). The IgG subclasses of myeloperoxidase (MPO)‐ANCA in both serum and renal tissue were measured and complement activation components were detected in serum. Results Patients in the concomitant group had both elevated serum IgG4 levels and positive MPO‐ANCA. They had higher levels of eosinophil counts, serum globulin, IgG, IgE and C‐reactive protein than patients in the AAV alone group. All 10 patients had glomerulonephritis with crescents and seven patients also had segmental necrosis of the glomerular capillary wall. Most of them also presented with storiform fibrosis and lymphoplasmacytic infiltration in renal interstitium with IgG4 positive plasma cells more than 10/high‐power field. Eight patients achieved remission with improved renal function, the other two patients were on maintenance dialysis. The IgG4 subclass of MPO‐ANCA was higher in the concomitant group than that in AAV alone group. A merge of IgG4 and MPO immunofluorescence was observed in parts of the mesangium of concomitant AAV and IgG4‐RD patients. For complement components, Bb and mannose‐binding lectin were elevated in serum of concomitant AAV and IgG4‐RD patients. Conclusion We showed a new overlap syndrome of AAV and IgG4‐RD, in which the IgG4 subclass of ANCA may be a pathogenic factor.
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