Matic G, Michelsen A, Hofmann D, Winkler R, Tiess M, Schneidewind J M, Müller W, Ramlow W
Labor Müller, Rostock, Germany.
Ther Apher. 2001 Feb;5(1):68-72. doi: 10.1046/j.1526-0968.2001.005001068.x.
Wegener's granulomatosis is a vasculitic disease predominantly affecting the upper respiratory tract, lungs, and kidneys. Three patients with Wegener's granulomatosis and rapidly progressive glomerulonephritis were treated with an intensified regimen of immunoadsorption (IA) (Excorim or Therasorb) in addition to cyclophosphamide (CYC) and methylprednisolone (PRE). Patient A had been in remission under oral CYC/PRE. The first exacerbation was treated successfully with 4 IA treatments without changing medication. Patient B experienced 3 flares within 1 year, which were treated with 28 IA (3-7 IAs/course), intravenous CYC after each course, and PRE. A fall of creatinine levels from 120 to 190 micromol/L to 100 micromol/L was noted after IA and before administration of CYC. Patient C presented in uremia. Autoantibodies were eliminated by 11 IA treatments parallel to CYC/PRE therapy. They remained within a normal range for >1 year's follow-up; however, kidney function did not return. In conclusion, the observations in Patients A and B suggest a beneficial therapeutic effect of early IA in WG.
韦格纳肉芽肿病是一种主要累及上呼吸道、肺和肾脏的血管炎性疾病。三名患有韦格纳肉芽肿病和快速进展性肾小球肾炎的患者,除接受环磷酰胺(CYC)和甲泼尼龙(PRE)治疗外,还采用了强化免疫吸附(IA)方案(Excorim或Therasorb)进行治疗。患者A在口服CYC/PRE治疗下处于缓解期。首次病情加重时,在未改变用药的情况下,通过4次IA治疗成功控制。患者B在1年内病情发作3次,接受了28次IA治疗(每次疗程3 - 7次IA),每次疗程后进行静脉注射CYC,并联合PRE治疗。IA治疗后且在给予CYC之前,肌酐水平从120至(190)微摩尔/升降至100微摩尔/升。患者C就诊时已处于尿毒症期。在接受CYC/PRE治疗的同时,通过11次IA治疗清除了自身抗体。在超过1年的随访期内,自身抗体水平一直保持在正常范围内;然而,肾功能并未恢复。总之,对患者A和B的观察结果表明,早期IA治疗对韦格纳肉芽肿病具有有益的治疗效果。