Pan C G, Strife C F, Ward M K, Spitzer R E, McAdams A J
Department of Pediatrics, Medical College of Wisconsin, Milwaukee.
Am J Kidney Dis. 1992 Jun;19(6):526-31. doi: 10.1016/s0272-6386(12)80830-4.
Hereditary angioedema (HAE) is characterized by a deficiency in C1 inhibitor protein (C1 INH) and by clinical symptoms of episodic swelling of subcutaneous or mucosal tissue. It has rarely been reported in association with non-systemic lupus erythematosus (SLE) glomerulonephritis (GN). A recent report of two cases indicates the prognosis to be poor, with both patients progressing to chronic renal failure 8 and 20 years after diagnosis. This report describes the 5-year follow-up of a previously unreported case of an 8-year-old boy with HAE and non-SLE membranoproliferative glomerulonephritis (MPGN). The patient developed macroscopic hematuria, azotemia, and a vasculitic rash. Treatment included prednisone and cyclophosphamide, resulting in clinical improvement. The present report also summarizes the long-term follow-up of three previously reported cases of HAE and non-SLE GN, 25, 16, and 10 years after their initial presentation. Patients monitored for 25 and 16 years had MPGN and normal renal function and received no therapy. The third patient, monitored for 10 years, had segmental MPGN. This patient presented with urinary abnormalities and, after treatment with prednisone, had improvement in her hematuria. None of these four patients developed chronic renal failure. These observations indicate a variable outcome in patients with HAE and non-SLE GN.
遗传性血管性水肿（HAE）的特征是C1抑制蛋白（C1 INH）缺乏以及皮下或粘膜组织间歇性肿胀的临床症状。与非系统性红斑狼疮（SLE）肾小球肾炎（GN）相关的情况鲜有报道。最近一份关于两例病例的报告显示预后较差，两名患者在诊断后8年和20年都进展为慢性肾衰竭。本报告描述了一名8岁患有HAE和非SLE膜增生性肾小球肾炎（MPGN）的男孩病例的5年随访情况，该病例此前未被报道。该患者出现肉眼血尿、氮质血症和血管炎性皮疹。治疗包括使用泼尼松和环磷酰胺，临床症状有所改善。本报告还总结了之前报道的三例HAE和非SLE GN病例在首次出现症状后25年、16年和10年的长期随访情况。接受25年和16年监测的患者患有MPGN但肾功能正常，未接受治疗。第三名患者接受了10年监测，患有节段性MPGN。该患者出现尿液异常，经泼尼松治疗后血尿有所改善。这四名患者均未发展为慢性肾衰竭。这些观察结果表明，HAE和非SLE GN患者的预后情况各不相同。
