以肾脏损害为主要表现的小血管炎的诊治探讨

Diagnosis and Treatment of Antineutrophil Cytoplasmic Antibody-associated Small-vessel Vasculitis with Renal Invovlvement

目的探讨以肾脏损害为主要表现的小血管炎的早期诊断及适时治疗。方法对40例肾脏损害为主要表现的小血管炎患者的临床资料进行回顾性分析。结果40例患者中,pANCA阳性33例,cANCA阳性7例。20例经甲基强的松龙、环磷酰胺冲击治疗,病情稳定,肾功能恢复正常;13例经甲强龙、环磷酰胺冲击治疗配合血液透析,肾功能好转;3例需要维持性血透;2例死于心力衰竭;2例死于肺出血合并消化道出血。结论伴有肾脏损害的ANCA相关性小血管炎临床表现缺乏特异性,早期诊断与治疗是改善预后的关键。

Objective To investigate the early diagnosis and proper treatment of small-vessel vaseulitis with renal involvement.Methods Forty cases with ANCA-associated small-vessel vasenlitis with renal involvement were analyzed retrospectively.Results pANCA of 33 patients were positive,cANCA were positive in 7 patient.The combined bolus treatment with Methylprednisone（MP）and CTX or hemodialysis were effective,20 patients achieved better prognosis.Thirteen patients received transient hemodialysis,3 patients need maintain hemodialysis.Two cases died due to heart failure,2patients with severe renal damage died from profuse pulmonary and peptic hemorrhage.Conclusion The patients of ANCA -associated small-vessel vasculitis with renal involvement are lack of the specific clinical manifestation, early diagnosis and proper treatment for them play a key role in improving its prognosis.