Hong Shyh-Ming, Chen Yung-Chang, Hsueh Swei, Jenq Chang-Chyi, Fang Ji-Tseng, Yang Chih-Wei, Tian Ya-Chung
Department of Nephrology, Chang Gung Memorial Hospital, Taipei, Taiwan.
J Nephrol. 2009 Jan-Feb;22(1):164-70.
Adult-onset Henoch-Schönlein purpura (HSP) is a rare systemic vasculitis characterized by a leukocytoclastic vasculitis of small vessels with the deposition of IgA immune complexes involving skin, gastrointestinal tract, joints and kidneys. Antineutrophil cytoplasmic antibody (ANCA) detected by indirect immunofluorescence assay is commonly found in other vasculitic disorders but rarely discovered in HSP patients. ANCA with perinuclear pattern has hardly ever reported in HSP patients. The diagnostic importance of ANCA still remains controversial. In addition, the simultaneous presence of diabetic nephropathy and HSP is uncommon. We present a case of an adult patient with diabetic nephropathy and superimposed HSP, which resulted in acute renal failure. Perinuclear-pattern ANCA was detected in the acute phase of HSP but disappeared when the disease resolved. Further, we have reviewed ANCA-positive HSP in this article.
成人起病的过敏性紫癜（HSP）是一种罕见的系统性血管炎，其特征为小血管白细胞破碎性血管炎，伴有IgA免疫复合物沉积，累及皮肤、胃肠道、关节和肾脏。通过间接免疫荧光法检测的抗中性粒细胞胞浆抗体（ANCA）在其他血管炎疾病中常见，但在HSP患者中很少发现。核周型ANCA在HSP患者中几乎从未有过报道。ANCA的诊断重要性仍存在争议。此外，糖尿病肾病与HSP同时存在并不常见。我们报告一例患有糖尿病肾病并叠加HSP的成年患者，该患者导致了急性肾衰竭。在HSP急性期检测到核周型ANCA，但疾病缓解时消失。此外，我们在本文中对ANCA阳性的HSP进行了综述。
