What is the mortality and morbidity associated with immunoglobulin A deficiency (IgAD)?

Updated: May 15, 2018
  • Author: Marina Y Dolina, MD; Chief Editor: Michael A Kaliner, MD  more...
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Answer

IgAD is more frequent in adult subjects with chronic lung disease than in a healthy, age-matched control subjects. [12]

The 20-year longitudinal study of healthy blood donors with incidental findings of IgAD used questionnaires and medical record reviews and found a 3-fold increase in rates of severe childhood respiratory conditions (9% vs 3%), a 4-fold increase in rates of severe adult respiratory conditions (16% vs 4%), a similar increase in recurrent mild respiratory tract infections, and a significant increase in rates of recurrent viral infections (16% vs 1%).

This study also noted a 4-fold increase in the rate of autoimmune conditions (23% in subjects with SIgAD vs 5% in control subjects); a 2.5-fold increase in the rate of abdominal symptoms caused by milk (16% vs 6%); and slight increases in the rates of atopic eczema (8% vs 5%), drug allergy (9% vs 5%), and food hypersensitivity (3% vs 1%). A slight decrease was observed in the rate of allergic rhinitis and/or eczema (11% vs 17%).

When IgAD is associated with one or more IgG subclass deficiencies or impaired polysaccharide responsiveness, some individuals with IgAD may develop recurrent sinopulmonary infections, especially in patients with concurrent IgG 2 and/or 4 subclass deficiency. This condition has been found to underlie some cases of familial deafness due to severe recurrent otitis. [42] GI tract infections and disorders in patients have been reported more frequently with absent secretory IgA, as has an increased incidence of cancer. Lack of secretory IgA has been hypothesized to compromise the defense against infection with Helicobacter pylori, which is thought to be a cause of stomach cancer.

The incidence of cancer among 562 Danish and Swedish subjects with CVID or IgA was compared with that of 2017 relatives for the period 1958-1996. Among 176 subjects with CVID, the incidence of cancer (all sites) was increased (standardized incidence ratio [SIR], 1.8; 95% confidence interval [CI], 1-2.9). Stomach cancer was increased (SIR, 10.3; 95% CI, 2.1-30.2), and malignant lymphoma was increased (SIR, 12.1; 95% CI, 3.3-31). Among 386 subjects with IgAD, the incidence of cancer (all sites) was not increased (SIR, 1); however, the incidence of stomach cancer was increased, albeit to an insignificant degree (SIR, 5.4; CI, 0.7-19.5). [43] The same study did not show an increase in lymphoid malignancies (non-Hodgkin lymphoma, Hodgkin disease) in IgAD subjects, even though some evidence in the literature indicates that the risk of developing a lymphoid malignancy is increased. [44]

A recent report described 63 Israeli children with SIgAD followed for 10 years, with malignancies diagnosed in 3 children (4.8%). [45]

Patients with IgAD who have a compensatory increase in IgM in their upper respiratory tract secretions and GI fluids tend to be less symptomatic. Note that patients with total IgAD are usually more symptomatic than patients partial IgAD.

  • A previously unrecognized clear association of SIgAD with recurrent parotitis of childhood (PTC) was demonstrated by Fazekas et al in an Austrian pediatric clinic population. [46] The prevalence of PTC in IgA-deficient patients (22%) was much higher than in a large population of healthy Austrian volunteers (0.3%). [40] Case reports from other countries support this association. [47]

  • Two studies provide conflicting evidence of oral conditions in children with SIgAD. A case-controlled (n = 34 and n = 111 [control group]) study of Hungarian children showed higher decayed, missing, and filled teeth and tooth surfaces in primary dentitions. The severities of mucosal or periodontal disorders were comparable with the normal population. [48] A smaller study of Iranian children (11 cases; 11 age- and sex-matched controls) showed no differences in findings including dental caries, plaque accumulation and periodontal status. [49] A more recent case-control study of 32 Icelandic adults with SIgAD compared with 63 randomly selected controls showed similar levels of periodontal health and dental health, but higher rates of tonsillectomy (44% vs 24%), adenoidectomy (31% vs 8%), and more pharyngitis, stomatitis, and herpes labialis. [50]


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