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Sections Immunoglobulin A Deficiency
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Medication

Medication Summary

Immunoglobulin A deficiency (IgAD) has no specific treatment. Replacement therapy is not practical for IgAD because of the short half-life of IgA and the relative paucity of IgA in commercial immunoglobulin preparations.

Antibiotic therapy is the first line of treatment, specific for sinopulmonary or GI tract infections. Associated sinopulmonary infections are treated according to treatment protocols used for community-acquired respiratory tract infections in healthy persons, but prolonged treatment may be necessary.

Immunization with pneumococcal and other polysaccharide vaccines is important; however, not all patients are able to mount an immune response. Postvaccination IgG titers can be obtained to confirm the presence of an age-appropriate protective level of antipneumococcal IgG. Patients with common variable immunodeficiency (CVID) or more subtle specific antibody deficiencies may be unable to mount a response to polysaccharide antigens; therefore, pneumococcal vaccination in CVID patients is often ineffective.

Use of IGIV as replacement therapy is not indicated for selective IgAD per se. In selected circumstances in patients with concomitant SIgAD and selective IgG antibody deficiency who have recurrent or chronic high-grade sinopulmonary infections, a trial of IGIV may be given to see if a substantial clinical response occurs. Most patients with IgAD as part of CVID and/or with concomitant specific IgG antibody deficiency can safely receive intravenous (IV) or subcutaneous (SC) IgG replacement therapy.^{[36, 81]}

Patients with known or possible anti-IgA antibodies are still at increased risk of anaphylaxis or severe IgG-mediated reactions.

Precautions must be used in the administration of IV immunoglobulin and other blood products in patients with IgAD because IV immunoglobulin preparations and other blood products contain at least small amounts of IgA.

Class Summary

Used to induce active immunity.

Pneumococcal vaccine polyvalent (PPV23; Pneumovax 23; Pnu-Imune 23)

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Contains capsular polysaccharides of 23 pneumococcal types, which comprise 98% of pneumococcal disease isolates. For use in children >2 y and adults at increased risk of pneumococcal disease and its complications because of other underlying health conditions. Also benefits adults ≥ 65 y.

Pneumococcal vaccine heptavalent (PCV7; Prevnar)

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Pneumococcal conjugate vaccine approved for infants and toddlers. Contains 7 purified capsular polysaccharides of S pneumoniae serotypes, accounting for 71% of infection among children < 24 m, each coupled with a nontoxic variant of diphtheria toxin, CRM 197.

Licensed for use in infants and young children in Feb 2000. Recommended for children aged 2-23 mo and for children aged 24-59 mo who are at increased risk for pneumococcal disease (eg, with sickle cell disease, HIV infection, other immunocompromising or chronic medical conditions). Licensed for infants aged ≥ 6 wk.

Follow-up

Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005 May. 94(5 Suppl 1):S1-63. [QxMD MEDLINE Link].
Daele J, Zicot AF. Humoral immunodeficiency in recurrent upper respiratory tract infections. Some basic, clinical and therapeutic features. Acta Otorhinolaryngol Belg. 2000. 54(3):373-90. [QxMD MEDLINE Link].
Wang N, Hammarström L. IgA deficiency: what is new?. Curr Opin Allergy Clin Immunol. 2012 Dec. 12(6):602-8. [QxMD MEDLINE Link].
Spickett GP, Misbah SA, Chapel HM. Primary antibody deficiency in adults. Lancet. 1991 Feb 2. 337(8736):281-4. [QxMD MEDLINE Link].
Longhurst HJ, O'Grady C, Evans G, De Lord C, Hughes A, Cavenagh J, et al. Anti-D immunoglobulin treatment for thrombocytopenia associated with primary antibody deficiency. J Clin Pathol. 2002 Jan. 55(1):64-6. [QxMD MEDLINE Link].
Hammarstrom L, Vorechovsky I, Webster D. Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID). Clin Exp Immunol. 2000 May. 120(2):225-31. [QxMD MEDLINE Link].
Vorechovsky I, Webster AD, Plebani A, Hammarstrom L. Genetic linkage of IgA deficiency to the major histocompatibility complex: evidence for allele segregation distortion, parent-of-origin penetrance differences, and the role of anti-IgA antibodies in disease predisposition. Am J Hum Genet. 1999 Apr. 64(4):1096-109. [QxMD MEDLINE Link].
Vorechovsky I, Blennow E, Nordenskjold M, et al. A putative susceptibility locus on chromosome 18 is not a major contributor to human selective IgA deficiency: evidence from meiotic mapping of 83 multiple-case families. J Immunol. 1999 Aug 15. 163(4):2236-42. [QxMD MEDLINE Link].
Pan-Hammarstrom Q, Salzer U, Du L, et al. Reexamining the role of TACI coding variants in common variable immunodeficiency and selective IgA deficiency. Nat Genet. 2007 Apr. 39(4):429-30. [QxMD MEDLINE Link].
Koskinen S, Tolo H, Hirvonen M, Koistinen J. Long-term persistence of selective IgA deficiency in healthy adults. J Clin Immunol. 1994 Mar. 14(2):116-9. [QxMD MEDLINE Link].
Desar IM, Weemaes CM, van Deuren M, van der Meer JW. Reversible hypogammaglobulinaemia. Neth J Med. 2007 Nov. 65(10):381-5. [QxMD MEDLINE Link].
International Union of Immunological Societies. Primary immunodeficiency diseases. Report of an IUIS Scientific Committee. Clin Exp Immunol. 1999 Oct. 118 Suppl 1:1-28. [QxMD MEDLINE Link].
Sazama K. Reports of 355 transfusion-associated deaths: 1976 through 1985. Transfusion. 1990 Sep. 30(7):583-90. [QxMD MEDLINE Link].
Rogers RL, Javed TA, Ross RE, et al. Transfusion management of an IgA deficient patient with anti-IgA and incidental correction of IgA deficiency after allogeneic bone marrow transplantation. Am J Hematol. 1998 Apr. 57(4):326-30. [QxMD MEDLINE Link].
Sanz C, Freire C, Ordinas A, Pereira A. An enzyme-linked immunosorbent assay applicable to screen blood donors for IgA deficiency. Haematologica. 1999 Oct. 84(10):887-90. [QxMD MEDLINE Link].
Thibault L, Beausejour A, de Grandmont MJ, Long A, Goldman M, Chevrier MC. Establishment of an immunoglobulin A-deficient blood donor registry with a simple in-house screening enzyme-linked immunosorbent assay. Transfusion. 2006 Dec. 46(12):2115-21. [QxMD MEDLINE Link].
Kaneko H, Suzuki H, Kondo N. [IgA subclass and IgA deficiency]. Nihon Rinsho Meneki Gakkai Kaishi. 2009 Jun. 32(3):142-8. [QxMD MEDLINE Link].
Giambra V, Cianci R, Lolli S, et al. Allele *1 of HS1.2 enhancer associates with selective IgA deficiency and IgM concentration. J Immunol. 2009 Dec 15. 183(12):8280-5. [QxMD MEDLINE Link].
Asano T, Kaneko H, Terada T, et al. Molecular analysis of B-cell differentiation in selective or partial IgA deficiency. Clin Exp Immunol. 2004 May. 136(2):284-90.
Castigli E, Geha RS. TACI, isotype switching, CVID and IgAD. Immunol Res. 2007. 38(1-3):102-11. [QxMD MEDLINE Link].
Castigli E, Wilson SA, Garibyan L, et al. TACI is mutant in common variable immunodeficiency and IgA deficiency. Nat Genet. 2005 Aug. 37(8):829-34. [QxMD MEDLINE Link].
Hummelshoj L, Ryder LP, Nielsen LK, Nielsen CH, Poulsen LK. Class switch recombination in selective IgA-deficient subjects. Clin Exp Immunol. 2006 Jun. 144(3):458-66. [QxMD MEDLINE Link].
Jin R, Kaneko H, Suzuki H, Arai T, Teramoto T, Fukao T, et al. Age-related changes in BAFF and APRIL profiles and upregulation of BAFF and APRIL expression in patients with primary antibody deficiency. Int J Mol Med. 2008 Feb. 21(2):233-8. [QxMD MEDLINE Link].
Buckley RH. Immunodeficiency diseases. JAMA. 1992 Nov 25. 268(20):2797-806. [QxMD MEDLINE Link].
Cucca F, Zhu ZB, Khanna A, et al. Evaluation of IgA deficiency in Sardinians indicates a susceptibility gene is encoded within the HLA class III region. Clin Exp Immunol. 1998 Jan. 111(1):76-80. [QxMD MEDLINE Link].
Vorechovsky I, Cullen M, Carrington M, et al. Fine mapping of IGAD1 in IgA deficiency and common variable immunodeficiency: identification and characterization of haplotypes shared by affected members of 101 multiple-case families. J Immunol. 2000 Apr 15. 164(8):4408-16. [QxMD MEDLINE Link].
Vorechovsky I, Webster AD, Hammarstrom L. Mapping genes underlying complex disorders: progress on IgA deficiency and common variable immunodeficiency. Adv Exp Med Biol. 2001. 495:183-90. [QxMD MEDLINE Link].
Rose MA, Schubert R, Schmitt-Grohe S, Reichenbach J, Zielen S. Immunoglobulins and inflammatory cytokines in nasal secretions in humoral immunodeficiencies. Laryngoscope. 2006 Feb. 116(2):239-44. [QxMD MEDLINE Link].
Brandtzaeg P, Karlsson G, Hansson G, Peteruson B, Bjorkander J, Hanson LA. The clinical condition of IgA-deficient patients is related to the proportion of IgD- and IgM-producing cells in their nasal mucosa. Clinical & experimental Immunology. 1987/03. 67 Issue 3:626-636.
Bakri F, Brauer AL, Sethi S, Murphy TF. Systemic and mucosal antibody response to Moraxella catarrhalis after exacerbations of chronic obstructive pulmonary disease. J Infect Dis. 2002 Mar 1. 185(5):632-40. [QxMD MEDLINE Link].
Lieberman D, Ben-Yaakov M, Lazarovich Z, et al. Chlamydia pneumoniae infection in acute exacerbations of chronic obstructive pulmonary disease: analysis of 250 hospitalizations. Eur J Clin Microbiol Infect Dis. 2001 Oct. 20(10):698-704. [QxMD MEDLINE Link].
Chauhan S, Gupta MK, Goyal A, Dasgupta DJ. Alterations in immunoglobulin & complement levels in chronic obstructive pulmonary disease. Indian J Med Res. 1990 Aug. 92:241-5. [QxMD MEDLINE Link].
Pilette C, Durham SR, Vaerman JP, Sibille Y. Mucosal immunity in asthma and chronic obstructive pulmonary disease: a role for immunoglobulin A?. Proc Am Thorac Soc. 2004. 1(2):125-35.
Papadopoulou A, Mermiri D, Taousani S, Triga M, Nicolaidou P, Priftis KN. Bronchial hyper-responsiveness in selective IgA deficiency. Pediatr Allergy Immunol. 2005 Sep. 16(6):495-500. [QxMD MEDLINE Link].
Samolitis NJ, Hull CM, Leiferman KM, Zone JJ. Dermatitis herpetiformis and partial IgA deficiency. J Am Acad Dermatol. 2006 May. 54(5 Suppl):S206-9. [QxMD MEDLINE Link].
Gustafson R, Gardulf A, Granert C, et al. Prophylactic therapy for selective IgA deficiency. Lancet. 1997 Sep 20. 350(9081):865. [QxMD MEDLINE Link].
Oen K, Petty RE, Schroeder ML. Immunoglobulin A deficiency: genetic studies. Tissue Antigens. 1982 Mar. 19(3):174-82. [QxMD MEDLINE Link].
Chandran S, Khetan D, Chaudhary R, Misra R, Aggarwal A. Low prevalence of IgA deficiency in north Indian population. Indian J Med Res. 2006 May. 123(5):653-6. [QxMD MEDLINE Link].
Cipe FE, Dogu F, Güloglu D, Aytekin C, Polat M, Biyikli Z, et al. B-cell subsets in patients with transient hypogammaglobulinemia of infancy, partial IgA deficiency, and selective IgM deficiency. J Investig Allergol Clin Immunol. 2013. 23(2):94-100. [QxMD MEDLINE Link].
Weber-Mzell D, Kotanko P, Hauer AC, et al. Gender, age and seasonal effects on IgA deficiency: a study of 7293 Caucasians. Eur J Clin Invest. 2004 Mar. 34(3):224-8.
Leiva LE, Zelazco M, Oleastro M, Carneiro-Sampaio M, Condino-Neto A, Costa-Carvalho BT, et al. Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry. J Clin Immunol. 2007 Jan. 27(1):101-8. [QxMD MEDLINE Link].
Oxelius VA, Laurell AB, Lindquist B, et al. IgG subclasses in selective IgA deficiency: importance of IgG2-IgA deficiency. New Engl. J. Med. Jun 11, 1981. 304:1476-77. [QxMD MEDLINE Link].
Mellemkjaer L, Hammarstrom L, Andersen V, et al. Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined Danish and Swedish study. Clin Exp Immunol. 2002 Dec. 130(3):495-500. [QxMD MEDLINE Link].
Cunningham-Rundles C, Zhou Z, Mankarious S, Courter S. Long-term use of IgA-depleted intravenous immunoglobulin in immunodeficient subjects with anti-IgA antibodies. J Clin Immunol. 1993 Jul. 13(4):272-8. [QxMD MEDLINE Link].
Shkalim V, Monselize Y, Segal N, Zan-Bar I, Hoffer V, Garty BZ. Selective IgA deficiency in children in Israel. J Clin Immunol. 2010 Sep. 30(5):761-5. [QxMD MEDLINE Link].
Fazekas T, Wiesbauer P, Schroth B et al. selective IgA deficiency in children with recurrent parotitis of childhood. Pediatr Infect Dis J. 2005 May. 24(5):461-2.
Shkalim V, Monselise Y, Mosseri R, Finkelstein Y, Garty BZ. Recurrent parotitis in selective IgA deficiency. Pediatr Allergy Immunol. 2004 Jun. 15(3):281-3. [QxMD MEDLINE Link].
Tar I, Kiss C, Marodi L, Marton IJ. Oral and dental conditions of children with selective IgA deficiency. Pediatr Allergy Immunol. 2008 Feb. 19(1):33-6. [QxMD MEDLINE Link].
Nikfarjam J, Pourpak Z, Shahrabi M, Nikfarjam L, Kouhkan A, Moazeni M, et al. Oral manifestations in selective IgA deficiency. Int J Dent Hyg. 2004 Feb. 2(1):19-25. [QxMD MEDLINE Link].
Jorgensen GH, Arnlaugsson S, Theodors A, Ludviksson BR. Immunoglobulin A deficiency and oral health status: a case-control study. J Clin Periodontol. 2010 Jan. 37(1):1-8. [QxMD MEDLINE Link].
Yalcin E, Kiper N, Gocmen A, OzcelIk U, Dogru D, MisirligIl Z. Pigeon-breeder's disease in a child with selective IgA deficiency. Pediatr Int. 2003 Apr. 45(2):216-8. [QxMD MEDLINE Link].
Sennekamp J, Morr H, Behr J. Extrinsic allergic alveolitis with IgA deficiency. Eur J Med Res. 2004 Dec 22. 9(12):573-4. [QxMD MEDLINE Link].
Jorgensen GH, Ornolfsson AE, Johannesson A, et al. Association of immunoglobulin A deficiency and elevated thyrotropin-receptor autoantibodies in two Nordic countries. Hum Immunol. 2011 Feb. 72(2):166-72. [QxMD MEDLINE Link].
Barka N, Shen GQ, Shoenfeld Y, et al. Multireactive pattern of serum autoantibodies in asymptomatic individuals with immunoglobulin A deficiency. Clin Diagn Lab Immunol. 1995 Jul. 2(4):469-72. [QxMD MEDLINE Link].
Casquero A, Ramos A, Barat A, Mampaso F, Caramelo C, Egido J, et al. Recurrent acute postinfectious glomerulonephritis. Clin Nephrol. 2006 Jul. 66(1):51-3. [QxMD MEDLINE Link].
Betterle C, Lazzarotto F, Spadaccino AC, et al. Celiac disease in North Italian patients with autoimmune Addison's disease. Eur J Endocrinol. 2006 Feb. 154(2):275-9. [QxMD MEDLINE Link].
Bjorkander J, Hammarstrom L, Smith CI, et al. Immunoglobulin prophylaxis in patients with antibody deficiency syndromes and anti-IgA antibodies. J Clin Immunol. 1987 Jan. 7(1):8-15. [QxMD MEDLINE Link].
Chowdary P, Nair D, Davies N, Malde R, Gatt A. Anaphylactic reaction with prothrombin complex concentrate in a patient with IgA deficiency and anti-IgA antibodies. Blood Coagul Fibrinolysis. 2010 Dec. 21(8):764-5. [QxMD MEDLINE Link].
Steel C, Vaida S, Mets B. Case report: massive blood transfusion in a patient with immunoglobulin a deficiency undergoing cesarean delivery. Anesth Analg. 2010 Apr 1. 110(4):1088-90. [QxMD MEDLINE Link].
Robitaille N, Delage G, Long A, Thibault L, Robillard P. Allergic transfusion reactions from blood components donated by IgA-deficient donors with and without anti-IgA: a comparative retrospective study. Vox Sang. 2010 Aug 1. 99(2):136-41. [QxMD MEDLINE Link].
Cunningham-Rundles C, Brandeis WE, Pudifin DJ, et al. Autoimmunity in selective IgA deficiency: relationship to anti-bovine protein antibodies, circulating immune complexes and clinical disease. Clin Exp Immunol. 1981 Aug. 45(2):299-304. [QxMD MEDLINE Link].
Valletta E, Fornaro M, Pecori S, Zanoni G. Selective immunoglobulin A deficiency and celiac disease: let's give serology a chance. J Investig Allergol Clin Immunol. 2011. 21(3):242-4. [QxMD MEDLINE Link].
Friman V, Nowrouzian F, Adlerberth I, Wold AE. Increased frequency of intestinal Escherichia coli carrying genes for S fimbriae and haemolysin in IgA-deficient individuals. Microb Pathog. 2002 Jan. 32(1):35-42. [QxMD MEDLINE Link].
Bekou V, Büchau A, Flaig MJ, Ruzicka T, Hogardt M. Cutaneous infection by Mycobacterium haemophilum and kansasii in an IgA-deficient man. BMC Dermatol. 2011 Jan 26. 11:3. [QxMD MEDLINE Link]. [Full Text].
Sripanidkulchai R, Suphakunpinyo C, Jetsrisuparb C, Luengwattanawanich S. Thai girl with ring chromosome 18 (46XX, r18). J Med Assoc Thai. 2006 Jun. 89(6):878-81. [QxMD MEDLINE Link].
Sigstad HM, Stray-Pedersen A, Froland SS. Coping, quality of life, and hope in adults with primary antibody deficiencies. Health Qual Life Outcomes. 2005 May 4. 3:31. [QxMD MEDLINE Link].
Gomez-Carrasco JA, Barrera-Gomez MJ, Garcia-Mourino V, et al. Selective and partial IgA deficiency in an adolescent male with bronchiectasis. Allergol Immunopathol (Madr). 1994 Nov-Dec. 22(6):261-3. [QxMD MEDLINE Link].
Lantz A, Armstrong J, Truemper E, et al. Immunoglobulin deficiency in children with a sudden overwhelming infection. Ann Allergy Asthma Immunol. 2001 Jan. 86(1):55-8. [QxMD MEDLINE Link].
Chen SM, Sheu JN, Chen JP, Yang MH. Community-acquired Pseudomonas aeruginosa pneumonia complicated with loculated empyema in an infant with selective IgA deficiency. Acta Paediatr Taiwan. 2002 May-Jun. 43(3):157-61. [QxMD MEDLINE Link].
Koskinen S. Long-term follow-up of health in blood donors with primary selective IgA deficiency. J Clin Immunol. 1996 May. 16(3):165-70. [QxMD MEDLINE Link].
Anani W, Triulzi D, Yazer MH, Qu L. Relative IgA-deficient recipients have an increased risk of severe allergic transfusion reactions. Vox Sang. 2014 Sep 15. [QxMD MEDLINE Link].
Jorgensen GH, Gardulf A, Sigurdsson MI, Sigurdardottir ST, Thorsteinsdottir I, Gudmundsson S, et al. Clinical symptoms in adults with selective IgA deficiency: a case-control study. J Clin Immunol. 2013 May. 33(4):742-7. [QxMD MEDLINE Link].
Tachdjian R. Complex regional pain syndrome treated with intravenous immunoglobulin in a patient with common variable immune deficiency. Pain Ther. 2013 Dec. 2(2):129-34. [QxMD MEDLINE Link]. [Full Text].
de Laat PC, Weemaes CM, Bakkeren JA, et al. Familial selective IgA deficiency with circulating anti-IgA antibodies: a distinct group of patients?. Clin Immunol Immunopathol. 1991 Jan. 58(1):92-101. [QxMD MEDLINE Link].
Kirkeleit J, Ulvestad E, Riise T, Bratveit M, Moen BE. Acute suppression of serum IgM and IgA in tank workers exposed to benzene. Scand J Immunol. 2006 Dec. 64(6):690-8. [QxMD MEDLINE Link].
Booth JR, Munks R, Sokol RJ. Isolation of IgA1 from human serum by affinity chromatography using an immobilized extract of the albumin gland of Helix pomatia. Transfus Med. 1995 Jun. 5(2):117-21. [QxMD MEDLINE Link].
Oltean S, Epure A, Lindström K, Pardi C. Detection of anti-IgA antibodies using the particle gel immunoassay: a rapid test for increased patient safety. Blood Transfus. 2014 Jul. 12(3):334-9. [QxMD MEDLINE Link]. [Full Text].
Rusconi F, Panisi C, Dellepiane RM et al. Pulmonary and sinus disease in primary humoral immunodeficiencies with chronic productive cough. Arch Dis Child. 2003 Dec. 88(12):1101-5. [QxMD MEDLINE Link].
Hermans PE, Diaz-Buxo JA, Stobo JD. Idiopathic late-onset immunoglobulin deficiency. Clinical observations in 50 patients. Am J Med. 1976 Aug. 61(2):221-37. [QxMD MEDLINE Link].
Kohler P. Pulmonary manifestations and management of antibody deficiency in adults. Chest. 1984 Sep. 86(3 Suppl):24S-28S. [QxMD MEDLINE Link].
Sundin U, Nava S, Hammarstrom L. Induction of unresponsiveness against IgA in IgA-deficient patients on subcutaneous immunoglobulin infusion therapy. Clin Exp Immunol. 1998 May. 112(2):341-6. [QxMD MEDLINE Link].
Vassallo RR. Review: IgA anaphylactic transfusion reactions. Part I. Laboratory diagnosis, incidence, and supply of IgA-deficient products. Immunohematol. 2004. 20:226-33.
Sandler SG, Mallory D, Malamut D, Eckrich R. IgA anaphylactic transfusion reactions. Transfus Med Rev. 1995 Jan. 9(1):1-8. [QxMD MEDLINE Link].
Knight AK, Bingemann T, Cole L, Cunningham-Rundles C. Frequent false positive beta human chorionic gonadotropin tests in immunoglobulin A deficiency. Clin Exp Immunol. 2005 Aug. 141(2):333-7.
Winters JL, Moore SB, Sandness C, Miller DV. Transfusion of apheresis PLTs from IgA-deficient donors with anti-IgA is not associated with an increase in transfusion reactions. Transfusion. 2004 Mar. 44(3):382-5.
Dahlbom I, Olsson M, Forooz NK, Sjoholm AG, Truedsson L, Hansson T. Immunoglobulin G (IgG) anti-tissue transglutaminase antibodies used as markers for IgA-deficient celiac disease patients. Clin Diagn Lab Immunol. 2005 Feb. 12(2):254-8. [QxMD MEDLINE Link].
Lenhardt A, Plebani A, Marchetti F, et al. Role of human-tissue transglutaminase IgG and anti-gliadin IgG antibodies in the diagnosis of coeliac disease in patients with selective immunoglobulin A deficiency. Dig Liver Dis. 2004 Nov. 36(11):730-4. [QxMD MEDLINE Link].
Villalta D, Alessio MG, Tampoia M, et al. Diagnostic accuracy of IgA anti-tissue transglutaminase antibody assays in celiac disease patients with selective IgA deficiency. Ann N Y Acad Sci. 2007 Aug. 1109:212-20. [QxMD MEDLINE Link].
Wang N, Truedsson L, Elvin K, Andersson BA, Rönnelid J, Mincheva-Nilsson L, et al. Serological assessment for celiac disease in IgA deficient adults. PLoS One. 2014. 9(4):e93180. [QxMD MEDLINE Link]. [Full Text].
Aittoniemi J, Koskinen S, Laippala P, et al. The significance of IgG subclasses and mannan-binding lectin (MBL) for susceptibility to infection in apparently healthy adults with IgA deficiency. Clin Exp Immunol. 1999 Jun. 116(3):505-8. [QxMD MEDLINE Link].
Alaswad B, Brosnan P. The association of celiac disease, diabetes mellitus type 1, hypothyroidism, chronic liver disease, and selective IgA deficiency. Clin Pediatr (Phila). 2000 Apr. 39(4):229-31. [QxMD MEDLINE Link].
Arulanandam BP, Raeder RH, Nedrud JG, et al. IgA immunodeficiency leads to inadequate Th cell priming and increased susceptibility to influenza virus infection. J Immunol. 2001 Jan 1. 166(1):226-31. [QxMD MEDLINE Link].
Badcock LJ, Clarke S, Jones PW, et al. Abnormal IgA levels in patients with rheumatoid arthritis. Ann Rheum Dis. 2003 Jan. 62(1):83-4. [QxMD MEDLINE Link].
Ballow M. Primary immunodeficiency disorders: antibody deficiency. J Allergy Clin Immunol. 2002 Apr. 109(4):581-91. [QxMD MEDLINE Link].
Braconier JH, Nilsson B, Oxelius VA, Karup-Pedersen F. Recurrent pneumococcal infections in a patient with lack of specific IgG and IgM pneumococcal antibodies and deficiency of serum IgA, IgG2 and IgG4. Scand J Infect Dis. 1984. 16(4):407-10. [QxMD MEDLINE Link].
Buckley RH. Advances in the diagnosis and treatment of primary immunodeficiency diseases. Arch Intern Med. 1986 Feb. 146(2):377-84. [QxMD MEDLINE Link].
Buckley RH. Primary Immunodeficiency Diseases. Middleton E Jr, Reed CE, Ellis EF, Adkinson NF Jr, Yunginger JW, Busse WW, eds. Allergy: Principles and Practice. 713-34.
Buckley RH. Primary immunodeficiency diseases: dissectors of the immune system. Immunol Rev. 2002 Jul. 185:206-19. [QxMD MEDLINE Link].
Buckley RH, Schiff RI. The use of intravenous immune globulin in immunodeficiency diseases. N Engl J Med. 1991 Jul 11. 325(2):110-7. [QxMD MEDLINE Link].
Burks AW, Sampson HA, Buckley RH. Anaphylactic reactions after gamma globulin administration in patients with hypogammaglobulinemia. Detection of IgE antibodies to IgA. N Engl J Med. 1986 Feb 27. 314(9):560-4.
Burrows PD, Cooper MD. IgA deficiency. Adv Immunol. 1997. 65:245-76. [QxMD MEDLINE Link].
Cardinale F, Friman V, Carlsson B, et al. Aberrations in titre and avidity of serum IgM and IgG antibodies to microbial and food antigens in IgA deficiency. Scand J Immunol. 1992 Aug. 36(2):279-83. [QxMD MEDLINE Link].
Carvalho Neves Forte W, Ferreira De Carvalho Junior F, Damaceno N, et al. Evolution of IgA deficiency to IgG subclass deficiency and common variable immunodeficiency. Allergol Immunopathol (Madr). 2000 Jan-Feb. 28(1):18-20. [QxMD MEDLINE Link].
Cataldo F, Marino V, Bottaro G, et al. Celiac disease and selective immunoglobulin A deficiency. J Pediatr. 1997 Aug. 131(2):306-8. [QxMD MEDLINE Link].
Cunningham-Rundles C. Clinical and immunologic analyses of 103 patients with common variable immunodeficiency. J Clin Immunol. 1989 Jan. 9(1):22-33. [QxMD MEDLINE Link].
Cunningham-Rundles C. Physiology of IgA and IgA deficiency. J Clin Immunol. 2001 Sep. 21(5):303-9. [QxMD MEDLINE Link].
Davies K, Stiehm ER, Woo P, Murray KJ. Juvenile idiopathic polyarticular arthritis and IgA deficiency in the 22q11 deletion syndrome. J Rheumatol. 2001 Oct. 28(10):2326-34. [QxMD MEDLINE Link].
Eckrich RJ, Mallory DM, Sandler SG. Laboratory tests to exclude IgA deficiency in the investigation of suspected anti-IgA transfusion reactions. Transfusion. 1993 Jun. 33(6):488-92. [QxMD MEDLINE Link].
French MA, 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. 1986 Dec. 66(3):640-7. [QxMD MEDLINE Link].
Friman V, Hanson LA, Bridon JM, et al. IL-10-driven immunoglobulin production by B lymphocytes from IgA- deficient individuals correlates to infection proneness. Clin Exp Immunol. 1996 Jun. 104(3):432-8. [QxMD MEDLINE Link].
Gutierrez MG, Kirkpatrick CH. Progressive immunodeficiency in a patient with IgA deficiency. Ann Allergy Asthma Immunol. 1997 Oct. 79(4):297-301. [QxMD MEDLINE Link].
Hahn DL. Chlamydia pneumoniae, asthma, and COPD: what is the evidence?. Ann Allergy Asthma Immunol. 1999 Oct. 83(4):271-88, 291; quiz 291-2. [QxMD MEDLINE Link].
Hanson LA, Bjorkander J, Carlsson B, et al. The heterogeneity of IgA deficiency. J Clin Immunol. 1988 May. 8(3):159-62. [QxMD MEDLINE Link].
Hanson LA, Soderstrom R, Nilssen DE, et al. IgG subclass deficiency with or without IgA deficiency. Clin Immunol Immunopathol. 1991 Nov. 61(2 Pt 2):S70-7. [QxMD MEDLINE Link].
Iizuka M, Itou H, Sato M, Yukawa M, Shirasaka T, Chiba M, et al. Crohn's disease associated with selective immunoglobulin a deficiency. J Gastroenterol Hepatol. 2001 Aug. 16(8):951-2. [QxMD MEDLINE Link].
Jones AL, Webb DJ. Selective IgA deficiency, hypothyroidism and congenital lymphoedema. Scott Med J. 1996 Feb. 41(1):22-3. [QxMD MEDLINE Link].
Kilic SS, Tezcan I, Sanal O, et al. Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases. Pediatr Int. 2000 Dec. 42(6):647-50. [QxMD MEDLINE Link].
Kinlen LJ, Webster AD, Bird AG, et al. Prospective study of cancer in patients with hypogammaglobulinaemia. Lancet. 1985 Feb 2. 1(8423):263-6. [QxMD MEDLINE Link].
Klemola T, Savilahti E, Arato A, et al. Immunohistochemical findings in jejunal specimens from patients with IgA deficiency. Gut. 1995 Oct. 37(4):519-23. [QxMD MEDLINE Link].
Koskinen S, Tolo H, Hirvonen M, Koistinen J. Long-term follow-up of anti-IgA antibodies in healthy IgA-deficient adults. J Clin Immunol. 1995 Jul. 15(4):194-8. [QxMD MEDLINE Link].
Kowalczyk D, Baran J, Webster AD, Zembala M. Intracellular cytokine production by Th1/Th2 lymphocytes and monocytes of children with symptomatic transient hypogammaglobulinaemia of infancy (THI) and selective IgA deficiency (SIgAD). Clin Exp Immunol. 2002 Mar. 127(3):507-12. [QxMD MEDLINE Link].
Kowalczyk D, Mytar B, Zembala M. Cytokine production in transient hypogammaglobulinemia and isolated IgA deficiency. J Allergy Clin Immunol. 1997 Oct. 100(4):556-62. [QxMD MEDLINE Link].
Kruszewska M, Kowalczyk D, Stopyrowa J, et al. Clinical manifestation of IgA deficiency. Rocz Akad Med Bialymst. 1995. 40(3):630-3. [QxMD MEDLINE Link].
Lilic D, Sewell WA. IgA deficiency: what we should-or should not-be doing. J Clin Pathol. 2001 May. 54(5):337-8. [QxMD MEDLINE Link].
Litzman J, Burianova M, Thon V, Lokaj J. Progression of selective IgA deficiency to common variable immunodeficiency in a 16 year old boy. Allergol Immunopathol (Madr). 1996 Jul-Aug. 24(4):174-6. [QxMD MEDLINE Link].
Marconi M, Plebani A, Avanzini MA, et al. IL-10 and IL-4 co-operate to normalize in vitro IgA production in IgA- deficient (IgAD) patients. Clin Exp Immunol. 1998 Jun. 112(3):528-32. [QxMD MEDLINE Link].
Matthews VB, Witt CS, French MA, et al. Central MHC genes affect IgA levels in the human: reciprocal effects in IgA deficiency and IgA nephropathy. Hum Immunol. 2002 May. 63(5):424-33. [QxMD MEDLINE Link].
Olerup O, Smith CI, Bjorkander J, Hammarstrom L. Shared HLA class II-associated genetic susceptibility and resistance, related to the HLA-DQB1 gene, in IgA deficiency and common variable immunodeficiency. Proc Natl Acad Sci U S A. 1992 Nov 15. 89(22):10653-7. [QxMD MEDLINE Link].
Ott MM, Ott G, Klinker H, Trunk MJ, Katzenberger T, Muller-Hermelink HK. Abdominal T-cell non-Hodgkin's lymphoma of the gamma/delta type in a patient with selective immunoglobulin A deficiency. Am J Surg Pathol. 1998 Apr. 22(4):500-6. [QxMD MEDLINE Link].
Oxelius VA, Carlsson AM, Hammarstrom L, et al. Linkage of IgA deficiency to Gm allotypes; the influence of Gm allotypes on IgA-IgG subclass deficiency. Clin Exp Immunol. 1995 Feb. 99(2):211-5. [QxMD MEDLINE Link].
Paul AC, Justus A, Balraj A, et al. Malignant otitis externa in an infant with selective IgA deficiency: a case report. Int J Pediatr Otorhinolaryngol. 2001 Aug 20. 60(2):141-5. [QxMD MEDLINE Link].
Prince HE, Norman GL, Binder WL. Immunoglobulin A (IgA) deficiency and alternative celiac disease- associated antibodies in sera submitted to a reference laboratory for endomysial IgA testing. Clin Diagn Lab Immunol. 2000 Mar. 7(2):192-6. [QxMD MEDLINE Link].
Reil A, Bein G, Machulla HK, et al. High-resolution DNA typing in immunoglobulin A deficiency confirms a positive association with DRB1*0301, DQB1*02 haplotypes. Tissue Antigens. 1997 Nov. 50(5):501-6. [QxMD MEDLINE Link].
Sanal O, Ersoy F, Metin A, Tezcan I, Berkel AI, Yel L. Selective IgA deficiency with unusual features: development of common variable immunodeficiency, Sjogren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura. Acta Paediatr Jpn. 1995 Aug. 37(4):526-9. [QxMD MEDLINE Link].
Schaffer FM, Palermos J, Zhu ZB, et al. Individuals with IgA deficiency and common variable immunodeficiency share polymorphisms of major histocompatibility complex class III genes. Proc Natl Acad Sci U S A. 1989 Oct. 86(20):8015-9. [QxMD MEDLINE Link].
Schroeder HW Jr, Schroeder HW 3rd, Sheikh SM. The complex genetics of common variable immunodeficiency. J Investig Med. 2004 Mar. 52(2):90-103. [QxMD MEDLINE Link].
Seager J, Jamison DL, Wilson J, et al. IgA deficiency, epilepsy, and phenytoin treatment. Lancet. 1975 Oct 4. 2(7936):632-5. [QxMD MEDLINE Link].
Sorensen RU, Hidalgo H, Moore C, Leiva LE. Post-immunization pneumococcal antibody titers and IgG subclasses. Pediatr Pulmonol. 1996 Sep. 22(3):167-73. [QxMD MEDLINE Link].
Steuer A, McCrea DJ, Colaco CB. Primary Sjogren's syndrome, ulcerative colitis and selective IgA deficiency. Postgrad Med J. 1996 Aug. 72(850):499-500. [QxMD MEDLINE Link].
Tangsinmankong N, Bahna SL, Good RA. The immunologic workup of the child suspected of immunodeficiency. Ann Allergy Asthma Immunol. 2001 Nov. 87(5):362-9; quiz 370, 423. [QxMD MEDLINE Link].
van de Kerkhof PC, Steijlen PM. IgA deficiency and psoriasis: relevance of IgA in the pathogenesis of psoriasis. Dermatology. 1995. 191(1):46-8. [QxMD MEDLINE Link].
Zenone T, Souquet PJ, Cunningham-Rundles C, Bernard JP. Hodgkin's disease associated with IgA and IgG subclass deficiency. J Intern Med. 1996 Aug. 240(2):99-102. [QxMD MEDLINE Link].

Media Gallery

Chest radiograph of a 50-year-old man with immunoglobulin A deficiency and severe bilateral pneumonia. He also had congenital heart disease. Serum immunoglobulin G and immunoglobulin M levels were normal.
Lateral chest radiograph of a 50-year-old man with immunoglobulin A deficiency and severe bilateral pneumonia.
Portable chest radiograph of a 50-year-old man with acute respiratory distress syndrome as a complication of severe bilateral pneumonia. The patient died from respiratory failure 2 days after this x-ray film was taken.

of 3

Author

Marina Y Dolina, MD Consulting Physician, Wellspan Lung, Sleep and Critical Care Consultants and Pulmonary Services, York Hospital, Wellspan Health System

Marina Y Dolina, MD is a member of the following medical societies: American College of Chest Physicians, American Medical Association, American Thoracic Society, Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Rebecca Bascom, MD, MPH Professor of Medicine, Department of Medicine, Division of Pulmonary, Allergy, and Critical Care Medicine, Pennsylvania State College of Medicine, Milton S Hershey Medical Center; Graduate Faculty Member, Pennsylvania State University College of Medicine and The Huck Institutes of the Life Sciences

Rebecca Bascom, MD, MPH is a member of the following medical societies: American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Michael R Simon, MD, MA Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Professor, Department of Internal Medicine, Oakland University William Beaumont University School of Medicine; Adjunct Staff, Division of Allergy and Immunology, Department of Internal Medicine, William Beaumont Hospital

Michael R Simon, MD, MA is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, American Federation for Medical Research, Michigan Allergy and Asthma Society, Michigan State Medical Society, Royal College of Physicians and Surgeons of Canada, Society for Experimental Biology and Medicine

Disclosure: Have a 5% or greater equity interest in: Secretory IgA, Inc. ; siRNAx, Inc.<br/>Received income in an amount equal to or greater than $250 from: siRNAx, Inc.

Chief Editor

Michael A Kaliner, MD Clinical Professor of Medicine, George Washington University School of Medicine; Medical Director, Institute for Asthma and Allergy

Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, Association of American Physicians

Disclosure: Nothing to disclose.

Additional Contributors

Melvin Berger, MD, PhD Adjunct Professor of Pediatrics and Pathology, Case Western Reserve University; Senior Medical Director, Clinical Research and Development, CSL Behring, LLC

Melvin Berger, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Clinical Investigation, Clinical Immunology Society

Disclosure: Received salary from CSL Behring for employment; Received ownership interest from CSL Behring for employment; Received consulting fee from America''s Health insurance plans for subject matter expert for clinical immunization safety assessment network acvtivity of cdc.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Bettina C Hilman, MD, to the development and writing of this article.

Sections Immunoglobulin A Deficiency
Overview
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References

Immunoglobulin A Deficiency Medication

Medication Summary

Vaccines, inactivated bacteria

Class Summary

Pneumococcal vaccine polyvalent (PPV23; Pneumovax 23; Pnu-Imune 23)

Pneumococcal vaccine polyvalent (PPV23; Pneumovax 23; Pnu-Imune 23)

Pneumococcal vaccine heptavalent (PCV7; Prevnar)

Pneumococcal vaccine heptavalent (PCV7; Prevnar)

Immunoglobulin A Deficiency Medication

Medication Summary

Vaccines, inactivated bacteria

Class Summary

Pneumococcal vaccine polyvalent (PPV23; Pneumovax 23; Pnu-Imune 23)

Pneumococcal vaccine polyvalent (PPV23; Pneumovax 23; Pnu-Imune 23)

Pneumococcal vaccine heptavalent (PCV7; Prevnar)

Pneumococcal vaccine heptavalent (PCV7; Prevnar)

References

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