Abstract
A 20-year health follow-up study of 159 initially healthy blood donors with a severe deficiency of serum IgA (<0.05×10−3 g/L) and of 45 donors with decreased serum IgA (0.05×10−3−0.8 g/L) was carried out. The findings indicate that persons with a severe deficiency of and decreased serum IgA who are healthy as young adults have an increased susceptibility to pneumonia and recurrent episodes of other respiratory infections and a higher risk of developing autoimmune diseases in middle age. Vitiligo, autoimmune hypothyreosis, milk intolerance, and possible rheumatoid arthritis were associated with severe IgA deficiency, but otherwise different degrees of IgA deficiency seem to be similar with respect to the appearance of diseases. Regardless of the fact that a total of 163 (80%) of the 204 IgA-deficient subjects had episodes of infections, drug allergy, or autoimmune or atopic disease, the finding of primary, selective IgA deficiency in a healthy adult per se does not seem to predict severe life-threatening illnesses at least during 20 years of life.
Similar content being viewed by others
References
Koistinen J: Selective IgA deficiency in blood donors. Vox Sang 29:192–202, 1975
Kanoh T, Mizumoto T, Yasuda N, Koya M, Ohno Y, Uchino H, Yoshimur K, Ohkubo Y, Yamaguchi H: Selective IgA deficiency in Japanese blood donors: frequency and statistical analysis. Vox Sang 50:81–86, 1986
Koskinen S, Tölö H, Hirvonen M, Koistinen J: Long-term persistence of selective IgA deficiency in healthy adults. J Clin Immunol 14:116–119, 1994
Ammann AJ, Hong R: Selective IgA deficiency: Presentation of 30 and a review of the literature. Medicine 50:223–236, 1971
Strober W, Sneller MC: IgA deficiency. Ann Allergy 66:363–375, 1991
Liblau RS, Bach J-F: Selective IgA deficiency and autoimmunity. Int Arch Allergy Immunol 99:16–27, 1992
Plebani A, Ugazio AG, Monafo V, Burgio GR: Clinical heterogeneity and reversibility of selective immunoglobulin A deficiency in 80 children. Lancet 1:829–831, 1986
Gordin A, Heinonen OP, Saarinen P, Lamberg B-A: Serumthyrotropin in symptomless autoimmune thyroiditis. Lancet 1:551–554, 1972
Aho K, Heliövaara M, Sievers K, Maatela J, Isomäki H: Clinical arthritis associated with positive radiological and serological findings in Finnish adults. Rheumatol Int 9:7–11, 1989
Savilahti E: IgA deficiency in children. Immunoglobulincontaining cells in the intestinal mucosa, immunoglobulins in secretions and serum IgA levels. Clin Exp Immunol 13:395–406, 1973
Hanson LÅ, Brandtzaeg P, Björkander J, Carlsson B, Griffiss JM, Mellander L, Karlsson G: IgA deficiency with and without mucosal infections. Adv Exp Med Biol 216B: 1449–1453, 1987
Mazanec MB, Nedrud JG, Kaetzel CS, Lamm ME: A three-tiered view of the role of IgA in mucosal defense. Immunol Today 14:430–5, 1993
Secretory IgA in recurrent urinary tract infections in childhood. Editorial. Lancet 2:433–434, 1988
Tuttle JP, Sarvas H, Koistinen J: The role of vaginal immunoglobulin A in girls with recurrent urinary tract infections. J Urol 120:742–744, 1978
De Laat PCJ, Weemaes CMR, Gonera R, van Munster PJJ, Bakkeren JAJM, Stoelinga GBA: Clinical manifestations in selective IgA deficiency in childhood. A follow-up report. Acta Paediat Scand 80:798–804, 1991
Hammarsröm L, Axelsson U, Björkander J, Hanson LÅ, Möller E, Smith CIE: HLA antigens in selective IgA deficiency. Distribution in healthy donors and patients with recurrent respiratory tract infections. Tissue Antigens 24:35–39, 1984
French MAH, Harrison G: An investigation into the effect of the IgG antibody system on the susceptibility of IgA-deficient patients to respiratory tract infections. Clin Exp Immunol 66:640–647, 1986
Burmester GR, Gramatzki M, von Gernler J, Bartels O, Kalden JR: Brief Communication: Pulmonary sarcoidosis associated with acquired humoral and cellular immunodeficiency. Clin Immunol Immunopathol 37:406–412, 1985
Rubinstein I, Baum GL, Hiss Y: Selective IgA deficiency and sarcoidosis. Chest 88:160, 1985
Collin P, Mäki M, Keyriläinen O, Hällström O, Reunala T, Pasternack A: Selective IgA deficiency and coeliac disease. Scand J Gastroenterol 27:367–371, 1992
Bader PI, Biegel A, Epinette WW, Nance WE: Vitiligo and dysgammaglobulinemia. A case report and family study. Clin Genet 7:62–76, 1975
Torrelo A, Espana A, Balsa J, Ledo A: Vitiligo and polyglandular autoimmune syndrome with selective IgA deficiency. Int J Dermatol 31:343–344, 1992
Eriksson S: Reversible IgA deficiency in hypothyroidism. Postgrad Med J 57:714–716, 1981
Seager J: IgA deficiency during treatment of infantile hypothyroidism with thyroxine. Br Med J 288:1562–1563, 1984
Herrero-Beaumont G, Armas JB, Elswood J, Will RK, Calin A: Selective IgA deficiency and spondyloarthropathy: A distinct disease? Ann Rheum Dis 49:636–637, 1990
McLean IL, Kidd BL, Thompson PW, Cawley M: Ankylosing spodylitis and selective IgA deficiency. Ann Rheum Dis 50:271, 1991
Camilleri JP, Moore RH, Griffiths DFR, Williams BD: Selective IgA deficiency associated with glomerulonephritis and oligoarthritis. Ann Rheum Dis 51:123–125, 1992
Plebani A, Monafo V, Ugazio AG, Monti C, Avanzini MA, Massimi P, Burgio GR: Comparison of the frequency of atopic diseases in children with severe and partial IgA deficiency. Int Arch Allergy Appl Immun 82:485–486, 1987
Ryser O, Morell A, Hitzig WH: Primary immunodeficiencies in Switzerland: First report of the national registry in adults and children. J Clin Immunol 8:479–485, 1988
Burgio GR, Duse M, Monafo V, Ascione A, Nespoli L: Selective IgA deficiency: Clinical and immunological evaluation of 50 pediatric patients. Eur J Pediat 133:101–106, 1980
Savilahti E, Pelkonen P, Visakorpi JK: IgA deficiency in children. A clinical study with special reference to intestinal findings. Arch Dis Child 46:665–670, 1971
Quigley EMM, Carmichael HA, Watkinson G: Adult celiac disease (celiac sprue), pernicious anemia and IgA deficiency. J Clin Gastroenterol 8:277–281, 1986
Falchuk KR, Falchuk ZM: Selective Immunoglobulin A deficiency, ulcerative colitis, and gluten-sensitive enteropathy—a unique association. Gastroentelogy 69:503–506, 1975
Collin P, Hällström O, Mäki M, Viander M, Keyriläinen O: Atypical coeliac disease found with serologic screening. Scand J Gastroenterol 25:245–250, 1990
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Koskinen, S. Long-term follow-up of health in blood donors with primary selective IgA deficiency. J Clin Immunol 16, 165–170 (1996). https://doi.org/10.1007/BF01540915
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01540915