Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy

Background Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon progressive or relapsing paralysing disease caused by inflammation of the peripheral nerves. If the hypothesis that it is due to autoimmunity is correct, removal of autoantibodies in the blood by plasma exchange should be beneficial. Objectives To evaluate the efficacy of plasma exchange in CIDP. Search methods We searched the Cochrane Neuromuscular Disease Group Specialized Register (14 May 2012), CENTRAL (2012, Issue 4), MEDLINE (January 1966 to May 2012), EMBASE (January 1980 to May 2012), CINAHL Plus (January 1937 to May 2012) and LILACS (January 1982 to May 2012). We also scrutinised the bibliographies of the trials, and contacted the trial authors and other disease experts. Selection criteria Randomised controlled trials (RCTs) or quasi-RCTs in participants of any age comparing plasma exchange with sham treatment or no treatment. Data collection and analysis Two authors selected the trials, extracted the data and assessed risk of bias independently. Where possible data were combined according to the methods of the Cochrane Neuromuscular Disease Review Group. Main results Primary outcome measure: one cross-over trial including 18 participants showed two (95% confidence interval (CI) 0.8 to 3.0) points more improvement after four weeks on an 11-point disability scale with plasma exchange (10 exchanges over four weeks) than with sham exchange. Rapid deterioration after plasma exchange occurred in eight of 12 who had improved. Secondary outcome measures: when the results of this trial and another with 29 participants treated in a parallel group design trial were combined, there were 31 points (95% CI 16 to 45, maximum score 280) more improvement in an impairment scale after plasma exchange (six exchanges over three weeks) than after sham exchange. There were significant improvements in both trials in an electrophysiological measure, the proximally evoked compound muscle action potential, after three or four weeks. Non-randomised evidence indicates that plasma exchange induces adverse events in 3% to 17% of procedures. These are sometimes serious. A trial showing no significant difference in the benefit between plasma exchange and intravenous immunoglobulin has been included in the Cochrane review of intravenous immunoglobulin for this condition. Authors' conclusions Moderate to high quality evidence from two small trials showed that plasma exchange provides significant short-term improvement in disability, clinical impairment and motor nerve conduction velocity in CIDP but rapid deterioration may occur afterwards. Adverse events related to difficulty with venous access, use of citrate and haemodynamic changes are not uncommon. More research is needed to identify agents which will prolong the beneficial action of plasma exchange.