以免疫吸附血漿析離療法治療重症肌無力症之評估

Immunoadsorption Plasmapheresis Therapy for Myasthenia Gravis

廣泛型重症肌無力(MG)患者血漿內大部分皆有濃度高低不一之＂乙醞膽鹼受體抗體＂(AchRBb)。血漿析離療法可以使抗體急速下降，並使病情迅速恢復。五例重度MG病患（3男2女，平均年齡49.6歲）在接受胸腺切除術及免疫抑制療法控制不良前提下，接受免疫吸附血漿析離療法。Plasmaflo AP 05W為血漿分離管，Immusorba TR-350做血漿灌流時之免疫吸附管。每個療程採隔日連續5次治療。在整個治療療程前、後分別測定MG分數，AchRAb濃度，免疫球蛋白(Ig)濃度，血中生化及血球數值。IP治療後，5位病人之肌力全部進步，其中2位呼吸衰竭病人可以順利拔除氣管內管。在逐次免疫吸附血漿析離治療下，平均AchRAb濃度下降至治療前濃度之74.7%、52.6%、43.3%、35.8%及36.5% 0血清蛋白質分別下降：IgM 57.4±18%；IgG 49±14.5%；低密度脂蛋白33.6±24.8%；球蛋白28.2±28.2%；膽固醇23.5±10.2%； IgA 21.3±31.8%；及白蛋白20.8±12%。治療中除一例出現導管感染外，未見其他相關併發症o總結，免疫吸附血漿析離療法對重度MG且傳統療法反應不佳之頑固型個案是一有效且安全的治療。

Circulating antibodies to the acetylcholine receptor(AchRAb)are detectable in most patients with generalized myasthenia gravis(MG). Plasmapheresis has been shown to induce a decline in AchRAb titers accompanied by rapid clinical improvement.. In this study, immunoadsorption plasmapheresis(IP)was performed in five patients(3 men, 2 women；mean age 49.6 years) with advanced MG refractory to thymectomy and immunosuppressants. Plasmaflo AP 05W (Asahi Medical Co., Tokyo, Japan) was used as a plasma separator and Immusorba TR-350 (Asahi Medical Co., Tokyo, Japan) was used as an immunoadsorption column for plasma perfusion. Each Course of treatment consisted of five sessions of plasmapheresis on alternate days. MG scores, AchRAb titers, immunoglobulin (Ig) levels, biochemistry of plasma, and complete blood counts were assessed before and immediately after the entire course of treatment. Muscle strength improved after IP in all five patients and two patients with respiratory failure were successfully extubated. The mean AchRAb titer fell to 74.7%, 52.6%, 43.3%, 35.8%, and 36.5% of the original level after each session of IP. Serum concentrations of Igs, cholesterol, and proteins fell as follows: IgM, 57.4±18 %; IgG, 49±14.5 %, low density lipoprotein, 33.6±24.8 %; globulin, 28.2±28.2 %; cholesterol, 23.5±10.2 %; IgA, 21.3±31.8 %; and albumin, 20.8±12 %. None of the patients experienced adverse effects related to IP, except for one incidence of catheter infection. These results indicate that IP is an effective and safe treatment for refractory MG.