Table 1.

Summary of studies.

Drugs/Intervention	Study/Series		Number of participants enrolled	Number of participants improved/primary endpoint fulfilled	Notes
Steroids	Dyck et al. [1982]	Prednisone vs. no treatment	40	12	28 patients completed trial, n = 14 prednisone treated
Plasma exchange	Dyck et al. [1986]	Prospective double blinded	15	9	n = 15 (PE), n = 14 (sham)
	Hahn et al. [1996]	Double blind, sham controlled, crossover	18	12	15 participants completed trial
IVIg	Hughes et al. [2008]	Randomized, placebo-controlled	117	32	n = 59 IVIg, n = 58 placebo
Azathioprine	Dalakas and Engel [1981]	Case series	4	3	Steroid nonresponder
	McCombe et al. [1987]	Case series	7	4
Cyclophosphamide	Good et al. [1998]	Case series	15	11
	Gladstone et al. [2005]	Case series	5	4	Severe, refractory patients Long time follow up [range: 1.6–4.8 years]
Ciclosporine A	Matsuda et al. [2004]		7	7
	Barnett et al. [1998]	Case series	19	15	Retrospective, relapsing and progressive course, patients refractory to steroid, IVIg, azathioprine, cyclophosphamide, plasma exchange
Mycophenolate Mofetil	Gorson et al. [2004]	Case series	12	3	Retrospective, mean dose: 2.1 g/day
	Umapathi and Hughes [2002]	Case series	4	0	No reduction of steroids or other immunosuppressants
Rituximab	Benedetti et al. [2011]	Case series	13	9	Retrospective
Methotrexate	RMC Trial Group [2009]	Randomized, double-blind, controlled trial	60	28	Primary endpoint: 20% reduction of steroids or IVIg; n = 27 (MTX), n = 32 (Placebo)

MTX: methotrexate; PE: plasma exchange; IVIg: Intravenous immunoglobulin.