title: Autoimmunity in patients with selective IgA deficiency.
abstract: OBJECTIVE
  Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deticiency. Patients with SIgAD have a greater risk of concomitant autoimmune disorders than healthy individuals. The exact mechanism underlying the relationship between autoimmunity and SIgAD is not fully understood. The aim of this study was to evaluate potential associations between autoimmunity and specific clinical or immunological findings in patients with SIgAD.
  
  
  METHODS
  The study population comprised 57 symptomatic patients (65% males) with confirmed SIgAD who were referred to our center. Demographic data and history of autoimmunity were recorded both for patients and for their relatives. Comprehensive clinical and laboratory examinations were performed to investigate autoimmune complications in all the patients.
  
  
  RESULTS
  Autoimmune disorders were documented in 17 cases (29.8%; 9 males and 8 females). The most common manifestations were thyroiditis, vitiligo, and hemolytic anemia (3 cases each). Ten patients (17.5%) had a family history of autoimmunity. Significant associations were detected between autoimmunity and increased duration of follow-up (P = .003), serum level of IgM (P = .01), regulatory T-cell count (P = .03), and class-switched memory B-cell count (P = .01). Four cases of autoimmune SIgAD (23.5%) progressed to common variable immunodeficiency during the follow-up period (P = .006).
  
  
  CONCLUSIONS
  Autoimmune disorders, autoimmune cytopenia, and Ig subclass deficiency can lead to severe clinical manifestations in patients with SIgAD. Therefore, immunologists and pediatricians should be aware of these conditions.
authors.forename: H
authors.initials: H
authors.lastname: Abolhassani
authors.forename: B
authors.initials: B
authors.lastname: Gharib
authors.forename: S
authors.initials: S
authors.lastname: Shahinpour
authors.forename: S N
authors.initials: SN
authors.lastname: Masoom
authors.forename: A
authors.initials: A
authors.lastname: Havaei
authors.forename: B
authors.initials: B
authors.lastname: Mirminachi
authors.forename: N
authors.initials: N
authors.lastname: Arandi
authors.forename: B
authors.initials: B
authors.lastname: Torabi-Sagvand
authors.forename: H A
authors.initials: HA
authors.lastname: Khazaei
authors.forename: J
authors.initials: J
authors.lastname: Mohammadi
authors.forename: N
authors.initials: N
authors.lastname: Rezaei
authors.forename: A
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000293:Adolescent; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology*; D015551:Autoimmunity; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D017098:IgA Deficiency / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology*; D007075:Immunoglobulin M / Q000097:blood; D007156:Immunologic Memory; D015994:Incidence; D007492:Iran / Q000453:epidemiology; D018655:Lymphocyte Count; D008297:Male; D011237:Predictive Value of Tests; D015995:Prevalence; D011379:Prognosis; D012307:Risk Factors; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
chemical_list: D007075:Immunoglobulin M