Clutterbuck E J, Evans D J, Pusey C D
Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London.
Nephrol Dial Transplant. 1990;5(3):161-7. doi: 10.1093/ndt/5.3.161.
Churg-Strauss syndrome is part of the spectrum of systemic vasculitis, but can be distinguished from the other necrotising vasculitides on the basis of clinical and histological criteria. Renal involvement is not regarded as a prominent feature and is generally mild. We report a series of 19 patients with Churg-Strauss syndrome referred to the Hammersmith Hospital between 1976 and 1986. Renal involvement was common (84%) and often severe: three patients had nephrotic syndrome; four presented with serum creatinine greater than 500 mumol/l; and two required dialysis. Focal segmental glomerulonephritis was the predominant lesion on biopsy (85%), often with necrotising features and crescent formation. Extravascular granulomata and eosinophilic infiltrates were uncommon. Treatment with high-dose prednisolone was generally successful in milder cases, but was not always sufficient to halt the progression of more severe vasculitis. In these cases, additional immunosuppressive therapy with azathioprine, cyclophosphamide or plasma exchange was of benefit. Renal disease improved in 14 of 16 patients, ten of whom have maintained normal renal function on follow-up. Only one patient died, as a result of progressive cardiac disease.
变应性肉芽肿性血管炎是系统性血管炎的一种，但可根据临床和组织学标准与其他坏死性血管炎相鉴别。肾脏受累并非突出特征，通常较轻。我们报告了1976年至1986年间转诊至哈默史密斯医院的一系列19例变应性肉芽肿性血管炎患者。肾脏受累很常见（84%）且往往严重：3例患者患有肾病综合征；4例患者血清肌酐大于500μmol/L；2例患者需要透析。活检时局灶节段性肾小球肾炎是主要病变（85%），常伴有坏死特征和新月体形成。血管外肉芽肿和嗜酸性粒细胞浸润并不常见。高剂量泼尼松龙治疗在较轻病例中通常成功，但并不总是足以阻止更严重血管炎的进展。在这些病例中，加用硫唑嘌呤、环磷酰胺或血浆置换等免疫抑制治疗有益。16例患者中有14例肾病有所改善，其中10例在随访中维持了正常肾功能。仅1例患者因进行性心脏疾病死亡。
