title: Co-Occurrence of Chronic Spontaneous Urticaria with Immunoglobulin A Deficiency and Autoimmune Diseases.
abstract: BACKGROUND
  Immunoglobulin (Ig) A deficiency is a primary immunodeficiency in which autoimmunity is frequently observed. Thirty to fifty percent of patients with spontaneous chronic urticaria have autoantibodies that are able to cross-link FcεRI on mast cells and basophils.
  
  
  METHODS
  We investigated whether spontaneous chronic urticaria in patients with IgA deficiency meets the criteria for autoimmunity. Four patients were screened for positivity to a skin prick test and an autologous serum skin test and for the presence of other autoimmune diseases. Patient sera were tested for the ability to activate basophils and mast cells in vitro by measuring surface CD63 expression and β-hexosaminidase release, respectively.
  
  
  RESULTS
  The autologous serum test was positive in all patients, and patient sera were found to induce CD63 upregulation on basophils and degranulation of an LAD2 mast cell line. Moreover, all patients were affected by other autoimmune disorders.
  
  
  CONCLUSIONS
  For the first time, these data point out chronic autoimmune urticaria in subjects with an IgA deficiency and confirm that different autoimmune disorders are common among patients with an IgA deficiency. Patients with chronic autoimmune spontaneous urticaria should be screened for IgA deficiency, especially if they are affected by other autoimmune disorders. Thus, spontaneous urticaria could mirror more complex systemic diseases, such as immune deficiency.
authors.affiliation: Dipartimento di Scienze Mediche e Biologiche, Universitx00E0; degli Studi di Udine, Udine, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Frossi
authors.forename: Stefano
authors.initials: S
authors.lastname: De Carli
authors.forename: Fleur
authors.initials: F
authors.lastname: Bossi
authors.forename: Carlo
authors.initials: C
authors.lastname: Pucillo
authors.forename: Marco
authors.initials: M
authors.lastname: De Carli
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology* / Q000378:metabolism; D001491:Basophils / Q000276:immunology / Q000378:metabolism; D015550:Cell Degranulation / Q000276:immunology; D005260:Female; D006801:Humans; D017098:IgA Deficiency / Q000150:complications* / Q000276:immunology* / Q000378:metabolism; D007073:Immunoglobulin E / Q000097:blood / Q000276:immunology; D008297:Male; D008407:Mast Cells / Q000276:immunology / Q000378:metabolism; D008875:Middle Aged; D017455:Receptors, IgE / Q000378:metabolism; D014581:Urticaria / Q000150:complications* / Q000276:immunology* / Q000378:metabolism; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D017455:Receptors, IgE; D007073:Immunoglobulin E