Table I.

Clinical characteristics of APS-1 patients.

Patient	Anti-IFNα4 (U)	VZV-IgG (EIU)	VZV	VZV reactivation	HSV	Unusual HSVa	Unusual primary VZVb	Hospitalizedc	Elispot	AIRE genotype	Age groupd
1	294	110	+		+		+			HMZ	50–59
2	195	69	+	+					+	HMZ	50–59
3	143	80	+	+	+	+				HMZ	30–39
4	141	64	+		+				+	HMZ	30–39
5	129	51	+				+	+		HTZ	20–29
6	122	37	+				+			HMZ	30–39
7	113	86	+	+						HTZ	30–39
8	112	33	+		+					HMZ	30–39
9	109	71	+		+					HMZ	>60
10	107	100	+							HTZ	10–19
11	103	68	+							HMZ	20–29
12	95	69	+		+	+		+	+	HMZ	30–39
13	95	80	+	+						HMZ	30–39
14	94	72	+		+				+	HMZ	40–49
15	94	51	+							HMZ	<10
16	94	72	+	+						HMZ	20–29
17	93	45	+	+				+		HMZ	40–49
18	92	77	+				+			HTZ	10–19
19	91	51	+	+	+	+	+	+		HMZ	40–49
20	91	85	+		+	+		+		HMZ	40–49
21	91	47	+	+						HMZ	>60
22	88	78	+							HMZ	>60
23	88	82	+		+	+			+	HTZ	20–29
24	88	70	+	+						HMZ	40–49
25	87	69	+				+		+	HMZ	40–49
26	87	84	+							HMZ	20–29
27	87	71	+		+					HMZ	40–49
28	85		+							HMZ	10–19
29	84	95	+		+	+	+	+		HMZ	50–59
30	84	72	+		+					HMZ	40–49
31	83	15	#							HTZ	10–19
32	83	64	+		+					HMZ	50–59
33	81		+						+	HTZ	50–59
34	75	58	+							HMZ	40–49
35	73	99	+		+					HMZ	30–39
36	66	86	+		+					HMZ	>60
37	61	99	+						+	HTZ	50–59
38	53	72	+						+	HTZ	50–59
39	50	81	+		+					HMZ	50–59
40	47		+				+			HMZ	10–19
41	44	56	+		+				+	HMZ	30–39
42	41	105	+							HMZ	30–39
43	35		+				+			HTZ	10–19
44	6	79	+							HMZ	10–19

IFN, interferon, VZV, varicella zoster virus, HSV, herpes simplex virus, HMZ, homozygous for the variant c.769C > T in AIRE, HMZ, compound heterozygous for the variant c.769C > T in AIRE.

# Vaccinated.

^aHSV infection with prolonged or atypical symptoms or non-mucosal location.

^bVZV primary infection with hospitalization or patient reported severe blistering or high fever.

^cHospitalized either due to VZV primary infection or reactivation or HSV infection.

^dAge is given as age group to preserve anonymity.