Table2:

Summary of reported cases series of XIAP with colitis case.

Publication	n	Age (Years)	S.T.A.C	Anti-TNF-a	Colectomy	HSCT	Outcome
(Speckmann et al., 2013)	25 XIAP/6 IBD	1–18	5/6	2/6 1-Rem	3/6	4/6	4 well, 1 symp 1 died
(Pachlopnik Schmid et al., 2011)	30 XIAP/5 IBD	4–41	5/5	1/5	1/5	5-NK	2 symp 3 died
(Yang et al., 2012)	9 XIAP/2 IBD	0.3–0.5	1/1 1-NK	1/2 1-Rem	No	No	1 well 1 died
(Zeissig et al., 2015)	96 Ped IBD/4XIAP	0.7–16	3/3 1-NK	4/4	2/4	No	3 symp 1 died
(Dziadzio et al., 2015)	4 XIAP with IBD	0.1–12	4/4	1/4	2/4	2/4	2 died
(Aguilar et al., 2014)	17 XIAP with IBD	0.3–41	12/14 3-NK	10/14 3-NK	7/14 3-NK	2/14 3-NK	3 died
Individual case reports*	6 XIAP with IBD	0.1–3	6/6	5/6	3/6	4/6	2 died
Total	44	0.1–41	36/39 (92%)	24/41 (58%)	18/41 (44%)	12/36 (33%)	13/44 died (29%)

Colectomy-colectomy or other surgical interventions; NK-not known; n-number of patients; Rem- in remission; S.T.A.C-Steroids, tacrolimus, azathioprine, cyclosporine; symp –symptomatic;

^*individual case reports from table 1.