Table 1.

Immunogenicity of COVID-19 Vaccines in Patients With IEI

Author	Total number of patients with IEI	Vaccine	IEI diagnosis	Humoral immunity findings	Cellular immunity findings
Abo Helo et al40	17	BNT162B2	CVID	65%a	NR
Amodio et al41	21	BNT162B2	XLA, CVID, antibody deficiency	95% anti-spike RBD antibody, 86% anti-trimeric spike Ab	76%b
Antoli et al42	28	BNT162B2, mRNA-1273, ChAdOx1 nCoV-19, Ad26.COV2.S	CVID	71.4%	71%
Arroyo-Sanchez et al43	18	BNT162B2, mRNA-1273, ChAdOx1	CVID	83% any, 50% neutralizing	83%
Barmettler et al44	62	BNT162B2, mRNA-1273, Ad26.COV2.S	Primary antibody deficiency	59.7% after initial series; 14x higher after booster 2erologic response	NR
Barrios et al45	17	BNT162B2	CVID	70.5%	82%
Bergman et al46	78	BNT162B2	CVID, XLA, CID, “monogenic”	73%
Bradley et al47	1	BNT162B2	WAS	100%	100%
Delmonte et al36	81	BNT162B2, mRNA-1273, Ad26.COV2.S	SCID, APECED, CD40L, CID, CVID, FOXN1, hypogammaglobulinemia, MagT1, immune dysregulation, RAG, RALD, SASH3, STAT 3 LOF, STAT3 GOF, WAS, WHIM, XLA	85%	NR
Fernandez-Salinas et al38	33	BNT162B2	CVID	33%	NR
Goda et al48	30	ChAdOx1 BNT162b2 Booster	CVID	83% Any after booster 80% Neutralizing after mRNA booster	53% after ChAdOx1 83% after mRNA booster
Gupta et al49	10	BNT162B2	CVID	NR	Lower SARS-CoV-2 tetramer-specific CD8+ T cells, lower CD8+ granzyme+ perforin+ T cells
Hagin et al50	26	BNT162B2	XLA, hypogammaglobulinemia, STAT1 GOF, CVID, STAT3 LOF, NFKB1, Complement deficiency, IgG2 deficiency, CVID	69%	73%
Jalil, Abraham et al51	1	BNT162B2	CVID	100%	NR
Jalil, Rowane et al52	1	BNT162B2	MagT1	100%	NR
Oshiro et al53	1	Coronovac	XLA	0%	100%
Pham et al54	33	BNT162B2, mRNA-1273	Hypo/agammaglobulinemia, XLA, CVID, SAD, Good syndrome, CD40L, CTLA4 haploinsufficiency, PIK3R1 deficiency, ataxia telangiectasia, ATP6AP1 deficiency	48% anti RBD-specific Ab; 6% with ACE2 receptor blocking activity > 50%	77% with detectable T cell specificity
Ponsford et al55	156	BNT162B2, mRNA-1273, ChAdOx1	CVID, hypogammaglobulinemia, CID, SAD, DiGeorge, XLA, STAT1 GOF, APECED, CD40L, CGD, CTLA4, complement 2 deficiency, ADA2, IFNGR1, CHH, STAT3 LOF, idiopathic CD4+ T cell lymphopenia, WAS	67%	NR
Pulvirenti et al56	58	BNT162B2	CVID	34% post-vaccination; 100% post-infection and vaccination serologic response	1/9 after immunization, 0/3 convalescent and immunized
Romano et al57	5	BNT162B2, mRNA-1273	CVID	80%	NR
Salinas et al58	47	BNT162B2	CVID, XLA	20%	70% CVID, 83% XLA
Sauerwein et al59	73	BNT162B2	CVID, PAD	48% of CVID, 77% of PAD	CVID nonresponders have defective vaccine specific activation of CXCR5-negative CD4+ memory T cells and defective T follicular helper cells. CVID responders and PAD have intact vaccine specific activation of CXCR5-CD4+ memory T cells
Schulz-Cherry et al60	25	mRNA-1273, BNT162B2	SAD, subclass deficiency, CVID, “other”	73%	NR
Shields et al61	168	BNT162B2, ChAdOx1	CVID, PAD, SAD, XLA, hyper IgM, CID, thymoma	55%	46.20%
Squire et al62	11	BNT162B2, mRNA-1273	CVID, XLA, WAS, DiGeorge, hypogammaglobulinemia	91%	NR
van Leeuwen et al39	505	mRNA-1273	CVID, PAD, XLA, CID, phagocytic defects	RBD-specific binding antibodies: 98.3% IgG subclass deficiency; 100% undefined antibody deficiency, 100% phagocytic defect, 15% XLA, 91% CID, 81% CVID	88% overall, 67% in CVID

Abbreviations: APECED, autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy; CID, combined immunodeficiency; COVID-19, coronavirus disease 2019; CVID, common variable immunodeficiency; IEI, inborn errors of immunity; Ig, immunoglobulin; mRNA, messenger RNA; NR, not reported; PAD, primary antibody deficiency; RBD, receptor binding domain; SAD, specific antibody deficiency; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2; SCID, severe combined immunodeficiency; WAS, Wiskott-Aldrich syndrome; XLA, X-linked agammaglobulinemia.

^aPercentage of persons with detectable SARS-CoV-2 spike-specific antibodies.

^bPercentage of persons with detectable SARS-CoV-2 spike-specific T cells.