What is the prevalence of autoimmune disease in immunoglobulin A deficiency (IgAD)?

Updated: May 15, 2018
  • Author: Marina Y Dolina, MD; Chief Editor: Michael A Kaliner, MD  more...
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Autoimmune disease is reported in approximately 20% of patients with CVID and may be associated with IgAD.

  • Autoantibodies are often produced but may be difficult to detect. The sera of individuals with IgAD may contain various autoantibodies that cause no disease or cause myasthenia gravis or thyroid disease.

  • In Sweden, the prevalence of IgAD in thyrotropin-receptor autoantibody–seropositive individuals is 10 times higher than expected in the general population. [53]

  • Other selective case reports indicate an association between SIgAD and type 1 diabetes mellitus, vertigo, vitiligo, and alopecia.

  • Rheumatoid arthritis and systemic lupus erythematosus are the diseases most commonly connected with IgAD.

  • In a survey of serum specimens from 60 healthy subjects with SIgAD, 16 of 21 different autoantibody levels were higher in IgAD subjects than in healthy control subjects. [54]

  • A recent case report described 2 episodes of acute postinfectious glomerulonephritis, separated by 15 years, in a 33-year-old man with SIgAD. [55]

  • A northern Italian medical school described a series of 109 patients with autoimmune Addison disease (AAD), 2 of whom had SIgAD. The patients with AAD demonstrated a 12-fold higher prevalence than the local population. [56]

The prevalence rate of IgG anti-IgA antibodies among white persons with IgAD is 30-40%. In patients with combined IgA-IgG subclass 2 deficiency, the rate is 50-60%. In contrast, the prevalence of IgE antibodies against IgA is extremely low.

IgA-deficient patients with anti-IgA antibodies may develop severe reactions when they are transfused with blood components that contain IgA. In the rare cases of true anaphylaxis, these antibodies are typically of the IgE class; however, anti-IgA antibodies of the IgG isotype can also cause anaphylactic-type reactions. [57] Although anaphylactic reactions occur in 1 in 20,000-47,000 transfusions, they constitute one of the frequent nonhemolytic causes of transfusion-related mortality.

A recent case described a patient with SIgAD who developed an anaphylactic reaction to a prothrombin complex concentrate; the authors stated that product information did not list IgAD as a contraindication. [58] A recent case report described the successful management of an SIgAD patient who required massive blood transfusion during emergency cesarean delivery. [59] New data quantify the risk of allergic reactions in recipients of blood components containing anti-IgA (ie, blood donated by SIgAD donors). A Canadian donor database was cross-referenced with transfusion reaction records; the incidence of allergic reactions was 1.15% in the anti-IgA group and 2.04% in the group that did not receive anti–IgA-containing blood. [60]

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