1. Typical FS. Data from series of 5 or more patients.

Study ID / name	Treatment	N	n(%)asymptomatic 6m	n(%)asymptomatic 1m	n(%)ventilated 1m	n(%)dead 6m	n(%)fluct / relapse	? Adverse event
Cheng 2003	PE	3	2 (66%)	2 (66%)	0	1 (33%)	0	1 pneumonia (day 12)
	Natural history	4	3 (75%)	3 (75%)	0	1 (25%)	0	1 cardiac arrest (day 22)
Mori 2001, 2002, 2006	PE	23	22 (96%)	‐	‐	0	‐	‐
	IVIg	28	25 (89%)	‐	‐	0	‐	‐
	Natural history	41	39 (95%)	‐	‐	0	‐	‐
Mizoguchi 1998	PE	10	‐	1 (10%)	‐	‐	‐	‐
	Natural history	6	‐	0	‐	‐	‐	‐
Kobayashi 1983	Natural history	5	3 (60%)	2 (40%)	5 (100%)	0	0	0
Yuki 1996	Tryptophan gel column	11	11 (100%)	0	‐	0	‐	‐
J49 Mukai 1978	Prednisolone	1	1 (100%)	‐	1 (100%)	0	0	0
	Natural history	6	6 (100%)	‐	6 (100%)	0	0	0
Shuaib 1986	Natural history	7	7 (100%)	‐	0	0	0	0
Barontini 1983	Prednisolone	1	1 (100%)	0	0	0	0	0
	Natural history	5	5 (100%)	2 (40%)	1 (25%)	0	0	0
Al‐Din 1982	Prednisolone	1	1 (100%)	0	0	0	0	0
	Natural history	4	4 (100%)	0	1 (25%)	0	0	0
Yin 1985	Methylprednisolone	2	‐	‐	0	0	0	‐
	Natural history	5	‐	‐	0	0	0	‐
Yip1991	Prednisolone	3	3 (100%)		0	0	2	0
	Natural history	6	6 (100%)		0	0	0	0