Enríquez Ricardo, Sirvent Ana Esther, Amorós Francisco, Pérez Miguel, Matarredona Jaime, Reyes Adolfo
Nephrology Section, General Hospital of Elche, Spain.
J Nephrol. 2005 May-Jun;18(3):318-22.
We describe the association of crescentic membranoproliferative glomerulonephritis and hypocomplementemic urticarial vasculitis syndrome. A 39-year-old woman presented edema and proteinuria and later a non-pruritic urticarial rash. Laboratory results showed nephrotic syndrome, hypocomplementemia and positive anti-C1q antibodies. Skin biopsy disclosed leukocytoclastic vasculitis. Acute renal failure developed. Renal biopsy revealed crescentic membranoproliferative glomerulonephritis. She was treated with corticosteroids and cyclosphosphamide with improvement of the renal function and partial remission of the nephrotic syndrome. Afterwards the nephrotic syndrome relapsed, mycophenolate mofetil in monotherapy was administered with reduction in proteinuria. As far as we know only 3 cases, 2 in children and one in an adult, of crescentic glomerulonephritis and hypocomplementemic urticarial vasculitis syndrome have been reported. In our patient renal manifestations preceded urticarial lesions. We provide information on the evolution during a 42-month follow-up.
我们描述了新月体性膜增生性肾小球肾炎与低补体血症性荨麻疹性血管炎综合征的关联。一名39岁女性出现水肿和蛋白尿，随后出现非瘙痒性荨麻疹皮疹。实验室检查结果显示为肾病综合征、低补体血症及抗C1q抗体阳性。皮肤活检显示白细胞破碎性血管炎。急性肾衰竭发生。肾活检显示新月体性膜增生性肾小球肾炎。她接受了糖皮质激素和环磷酰胺治疗，肾功能改善，肾病综合征部分缓解。此后肾病综合征复发，给予霉酚酸酯单药治疗，蛋白尿减少。据我们所知，仅报道了3例新月体性肾小球肾炎与低补体血症性荨麻疹性血管炎综合征病例，2例为儿童，1例为成人。在我们的患者中，肾脏表现先于荨麻疹病变出现。我们提供了42个月随访期间的病情演变信息。
