Table 1.

Clinical characteristics of CVID patients with and without autoimmune disease

Pt	Age (yrs)	Sex	CVID main clinical features	Details if T cell dysfunction present	Autoimmune disease	Details on auto antibody testing	Infected at time of study	Associated disease features
1	41	F	Low IgG, IgA and IgM, Ab dysfunction and T cell dysfunction	Poor response to tetanus on T cell proliferation studies	Thyroiditis	Antithyroglobulin antibodies present	No	Bronchiectasis
2	39	M	Low IgG, IgA and IgM, Ab dysfunction and T cell dysfunction	Poor response to candida on T cell proliferation studies	Neutropenia, anemia	Bone marrow with increase ingranulocytes and a T-cell infiltrate suggestive of an autoimmune induced neutropenia; responsive to GCSF), direct Coombs positive anemia	No	Chronic thrush, Diarrhea (non infectious etiology to date)
3	55	F	Low IgG, IgA and IgM, Ab dysfunction		Thyroiditis	Antithyroglobulin antibodies present	No	Bronchiectasis
4	16	M	Low IgG, IgA and IgM, Ab dysfunction		Neutropenia	Antineutrophil antibodies present	No	Bronchiectasis, anemia (negative Coombs however, no autoAb to intrinsic factor or parietal cells performed to rule out pernicious anemia), nephrotic syndrome
5	8	M	Low IgG, IgA and IgM, Ab dysfunction and T cell dysfunction	Poor response to tetanus and candida on T cell proliferation studies	Anemia, inflammatory bowel disease (diagnosis by colonoscopy)	Direct Coombs positive anemia	No (stool culture, O&P, Giardia, and C. dificile all negative)
6	6	F	Low IgG, IgA and IgM, Ab dysfunction and T cell dysfunction	Poor response to tetanus and candida on T cell proliferation studies	Anemia, inflammatory bowel disease (no biopsy was performed)*	Direct Coombs positive anemia	No (stool culture, O&P, Giardia, and C. dificile all negative)
7	6	F	Low IgG, IgA and IgM, Ab dysfunction		Hepatitis, anemia	Direct Coombs positive anemia	No	Anemia also secondary to B- thalassemia trait
8	66	F	Low IgG, IgA and IgM, Ab dysfunction		Thyroiditis	Antithyroglobulin antibodies present	No	Bronchiectasis
9	21	M	Low IgG, IgA and IgM, Ab dysfunction		N/A	None detected	No	Bronchiectasis
10	31	F	Low IgG, IgA and IgM, Ab dysfunction		N/A	None detected	No	Bronchiectasis
11	11	M	Low IgG, IgA and IgM,, Ab dysfunction		N/A	None detected	No	Asthma, bronchiectasis
12	33	M	Low IgG, IgA and IgM,, Ab dysfunction		N/A	None detected	No	Bronchiectasis, asthma
13	67	M	Low IgG, IgA and IgM, Ab dysfunction		N/A	None detected	No	Bronchitis, sinusitis
14	21	M	Low IgG, IgA and IgM, Ab dysfunction		N/A	None detected	No	Gastritis, asthma, schizoaffective disorder
15	16	M	Bruton’s XLA		N/A	None detected	No	Right eye blindness due to CMV infection
16	35	M	Bruton’s XLA		N/A	None detected	No	Left lower leg paralysis due to polio infection in infancy

Note: No subject underwent splenectomy.