Table 2.

Treatment of chronic inflammatory demyelinating polyradiculoneuropathy based on evidence of clinical trials.

Treatment	Trial	Year	Trial design	Patients (n)	Outcome
Effective treatment compared to supportive care or placebo alone
Corticosteroids	Dyck et al.	1982	Prednisone (120 mg/2 d tapered to 0mg 12 weeks) (n=14) versus supportive care. Randomized controlled trial, no blinding	28	Significant better outcome in changes in NDS and nerve conduction studies after 12 weeks.
Plasma exchange	Dyck et al.	1986	PE (6x) versus sham-treatment, doubleblind	29	Significant better outcome in measurements of nerve conduction (total, motor, proximal, velocity, and amplitude) in the PE-group.
	Hahn et al.	1996	PE (10x) versus sham-treatment, doubleblind, crossover	18	Significant improvement in PE group in clinical outcome measures (NDS) and in most electrophysiological measurements.
IVIg, plasma exchange	Dyck et al.	1994	PE (7x) versus IVIG (0.4 g/kg, 1 per 3 weeks then 0.2 g/kg, 1 per 3 weeks) crossover in case of no improvement or worsening, single-blind	20	No significant differences between the two groups in clinical outcome (NDS) and electrophysiological parameter.
IVIg	Van Doorn et al.	1990	IVIg (0.4 g/kg, 5 d) versus placebo, randomized, double-blind, crossover trial	7	Clinical response in 7 of 7 patients after IVIg treatment.
IVIg	Vermeulen et al.	1993	IVIg 0.4 g/kg, 5 d (n=15) versus placebo (n=13), double-blind, parallel group randomized, controlled trial	28	No differences in clinical improvement defined as decrease on Rankin scale.
IVIg	Hahn et al. 1996	1996	IVIg (0.4 g/kg, 5 d) versus placebo, randomized, double-blind, crossover trial	25	Improvement in neurological scores (NDS); clinical grade (CG), grip strength (GS) after IVIg treatment.
IVIg	Mendell et al. 2001	2001	IVIg (1 g/kg on d 1, 2, 21) (n=30) versus placebo (n=23), randomized, controlled, double-blind	53	Improvement in primary outcome measure (change in muscle strength from baseline to day 42) and secondary outcome measures (vital capacity, nerve conduction studies)
IVIg, corticosteroids	Hughes et al.	2001	IVIg (2.0 g/kg) (n=12) versus prednisolone (60 mg/d 2 weeks, 40 mg/d, 1 week, 30 mg/d 1 week, 20 mg/d 1 week, 10 mg/d 1 week) (n=12), randomized, doubleblind, crossover trial	24	Significant improvement in changes in an 11-point disability scale after 2 weeks in both treatment arms.
IVIg	The ICE Trial	2008	IVIg (2 g/kg, 2–4 days then 1 g/kg every 3 weeks for up to 24 weeks) versus placebo, randomized, double-blind, placebo- controlled, response-conditional crossover trial. Two periods (responseconditional crossover (rescue) period, extension phase)	117	54% participants treated with IVIg improved in adjusted INCAT disability score that was maintained through to week 24 compared to 21% of patients who received placebo (p=0.0002). Longer time to relapse during the extension phase in IVIg treated patients (p=0.011).
Treatments with uncertain or no benefit
Azathioprine	Dyck et al.	1985	Azathioprine 2 mg/kg+prednisone (n=14) versus prednisone (n=13). Randomized trial, parallel group design, not blind.	27	No significant differences between the two groups after 9 months.
Interferonbeta 1a	Hadden et al.	1999	IFN-β (3 MIU for 2 weeks then 6 MIU for 10 weeks) s.c. 3xweekly versus placebo, controlled double-blind, crossover trial.	10	No significant difference between the treatment arms in any of clinical or neurophysiological measures.

PE, plasma exchange; IVIg, intravenous immunoglobulins; NDS, neurological disability score.