AlHermi Badriya, Al Mosawi Zakiya, Mohammed Deena
Department of Pediatrics, Salmaniya Medical Complex, Manama, Kingdom of Bahrain.
Saudi J Kidney Dis Transpl. 2017 Jul-Aug;28(4):929-933.
Hypocomplementic urticarial vasculitis syndrome (HUVS) is an autoimmune disease characterized by recurrent urticaria, arthritis, and glomerulonephritis (GN). Anti-C1q antibody is the marker of HUVS together with low levels of classical pathway complements which are C2, C3, C4, and C1q. We report a case of a 6-year-old boy who presented with episodes of rashes, injected conjunctiva, abdominal pain, and arthritis, diagnosed as HUVS. He had low C3, low CH50, normal C4, and positive C1q antibody. His urinalysis showed intermittent microscopic hematuria only. One year later, his laboratories showed persistent low C3 and positive Anti-ds DNA. The urinalysis showed hematuria, pyuria, and nephrotic-range proteinuria. Urine protein to creatinine ratio was 101.8 h mg/mmol. Kidney biopsy showed mesangioproliferative GN consistent with the diagnosis of HUVS. The patient was treated initially with prednisolone then azathioprine was added to the regimen. He showed good response with the disappearance of hematuria and proteinuria. Nine months later, he had no skin rashes with normal urinalysis and normal anti-ds DNA antibody. We report a case with HUVS and GN with positive anti-dsDNA antibody that revealed good response to combination of immunosuppressive therapy.
低补体血症性荨麻疹性血管炎综合征（HUVS）是一种自身免疫性疾病，其特征为反复出现荨麻疹、关节炎和肾小球肾炎（GN）。抗C1q抗体是HUVS的标志物，同时伴有经典途径补体（C2、C3、C4和C1q）水平降低。我们报告一例6岁男孩，其出现皮疹、结膜充血、腹痛和关节炎发作，被诊断为HUVS。他的C3降低、CH50降低、C4正常且C1q抗体阳性。他的尿液分析仅显示间歇性镜下血尿。一年后，他的实验室检查显示C3持续降低且抗双链DNA阳性。尿液分析显示血尿、脓尿和肾病范围蛋白尿。尿蛋白与肌酐比值为101.8 h mg/mmol。肾活检显示系膜增生性肾小球肾炎，符合HUVS的诊断。该患者最初接受泼尼松龙治疗，随后在治疗方案中加用硫唑嘌呤。他对治疗反应良好，血尿和蛋白尿消失。9个月后，他没有皮肤皮疹，尿液分析和抗双链DNA抗体均正常。我们报告一例伴有抗双链DNA抗体阳性的HUVS和肾小球肾炎患者，该患者对免疫抑制联合治疗反应良好。
