Chen T C, Chung F R, Lee C H, Huang S C, Chen J B, Hsu K T
Division of Nephrology, Department of Internal Medicine, Chang Gung Memorial Hospital Kaohsiung, Kaohsiung, Taiwan.
Clin Nephrol. 2004 Mar;61(3):213-6. doi: 10.5414/cnp61213.
Henoch-Schoenlein purpura (HSP) crescentic glomerulonephritis with acute renal failure in adults is extremely rare. The condition carries a grave renal outcome if it is not appropriately managed. Oral corticosteroids, intravenous methylprednisolone pulse therapy and plasmapheresis with concomitant plasma replacement have been used alone or in various combinations to treat patients with HSP nephritis, yet the effects are uncertain. We describe a 33-year-old man with oliguric acute renal failure in the setting of HSP crescentic glomerulonephritis that is refractory to intravenous methylprednisolone pulse therapy (1,000 mg/day for 3 days) with maintained oral prednisolone (1 mg/kg/day) and oral cyclophosphamide (2 mg/kg/day) for 3 weeks, resulting in successful recovery of renal function after 9 sessions of simple double-filtration plasmapheresis treatment without concomitant plasma replacement. There was no recurrence of vasculitic events within 18 months. In this case, we emphasize that simple double-filtration plasmapheresis without concomitant plasma replacement is an effective and safe modality therapy for adult patients with HSP crescentic glomerulonephritis and acute renal failure, especially when conventional therapy has failed.
成人过敏性紫癜(HSP)伴新月体性肾小球肾炎并急性肾衰竭极为罕见。若处理不当,该病症会导致严重的肾脏预后。口服糖皮质激素、静脉注射甲泼尼龙冲击疗法以及血浆置换联合血浆置换液输注已单独或联合用于治疗HSP肾炎患者,但效果尚不确定。我们报道一名33岁男性,患有HSP新月体性肾小球肾炎并少尿性急性肾衰竭,对静脉注射甲泼尼龙冲击疗法(1000mg/天,共3天)、持续口服泼尼松龙(1mg/kg/天)及口服环磷酰胺(2mg/kg/天)3周均无反应,在未联合血浆置换液输注进行9次单纯双重滤过血浆置换治疗后,肾功能成功恢复。18个月内未出现血管炎事件复发。在此病例中,我们强调,对于患有HSP新月体性肾小球肾炎并急性肾衰竭的成年患者,尤其是传统治疗无效时,单纯双重滤过血浆置换且不联合血浆置换液输注是一种有效且安全的治疗方式。