The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency

Selective immunoglobulin A deficiency (SIgAD) is the most common primary immunodeficiency, defined as an isolated deficiency of IgA (less than 0.07 g/L). Although the majority of people born with IgA deficiency lead normal lives without significant pathology, there is nonetheless a significant assoc...

Full description

Saved in:

Bibliographic Details
Published in:	Clinical reviews in allergy & immunology 2020-02, Vol.58 (1), p.107-133
Main Authors:	Odineal, David D., Gershwin, M. Eric
Format:	Article
Language:	eng
Subjects:	Allergology Anemia Ankylosing spondylitis Arthritis Atopy Autoimmune diseases Autoimmune hemolytic anemia Autoimmunity Celiac disease Development and progression Diabetes mellitus Diabetes mellitus (insulin dependent) Epidemiology Etiology Health aspects Health risks Hemolytic anemia Hepatitis Hyperthyroidism Hypothyroidism Immunodeficiency Immunoglobulin A Immunology Infection Internal Medicine Lichen planus Literature reviews Lupus Medicine Medicine & Public Health Mucosa Multiple sclerosis Neuromuscular junctions Primary immunodeficiencies Purpura Rheumatoid arthritis Rheumatoid factor Risk assessment Scleroderma Scleroderma (Disease) Systemic lupus erythematosus Systemic scleroderma Ulcerative colitis
Online Access:	Get full text
Tags:	Add Tag No Tags, Be the first to tag this record!

Description
Summary:	Selective immunoglobulin A deficiency (SIgAD) is the most common primary immunodeficiency, defined as an isolated deficiency of IgA (less than 0.07 g/L). Although the majority of people born with IgA deficiency lead normal lives without significant pathology, there is nonetheless a significant association of IgA deficiency with mucosal infection, increased risks of atopic disease, and a higher prevalence of autoimmune disease. To explain these phenomena, we have performed an extensive literature review to define the geoepidemiology of IgA deficiency and particularly the relative risks for developing systemic lupus erythematosus, hyperthyroidism, hypothyroidism, type 1 diabetes mellitus, Crohn’s disease, ulcerative colitis, rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, and vitiligo; these diseases have strong data to support an association. We also note weaker associations with scleroderma, celiac disease, autoimmune hepatitis, immune thrombocytopenic purpura, and autoimmune hemolytic anemia. Minimal if any associations are noted with myasthenia gravis, lichen planus, and multiple sclerosis. Finally, more recent data provide clues on the possible immunologic mechanisms that lead to the association of IgA deficiency and autoimmunity; these lessons are important for understanding the etiology of autoimmune disease.
ISSN:	1080-0549 1559-0267