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Specific antibody deficiency

INTRODUCTION

Specific antibody deficiency describes a deficient-specific antibody response to polysaccharide antigens in an individual with normal responses to protein antigens, normal serum levels of immunoglobulins, and normal IgG subclass concentrations.

This topic will discuss the epidemiology, pathogenesis, evaluation, diagnosis, and management of this disorder. A detailed discussion of the interpretation of vaccine responses and an overview of humoral immune deficiencies are presented elsewhere. (See "Assessing the immunologic response to vaccination" and "Primary humoral immune deficiencies: An overview".)

OVERVIEW

Specific antibody deficiency was first reported in a small group of patients in the early 1980s [1,2]. Subsequently, it was established that a deficient-specific antibody response to polysaccharide antigens can be seen in both children and adults [3-6]. It is one of the most commonly identified immune defects among patients presenting with recurrent and/or severe sinopulmonary infections. (See 'Epidemiology' below.)

Specific antibody deficiency may be identified as part of a host of distinct primary or secondary immunodeficiency disorders, such as common variable immune deficiency or asplenia, or it may exist in isolation, as the only identifiable immune problem [7-12].

Terminology — The terminology for specific antibody deficiency or polysaccharide nonresponse is not standardized, and other names appear in the literature, including "impaired polysaccharide responsiveness" and "selective antibody deficiency with normal immunoglobulins." In this review, specific antibody deficiency refers to the condition in which polysaccharide nonresponse is the only identifiable abnormality. We use the descriptive term "polysaccharide nonresponse" to denote the immunologic defect, which may occur in a variety of disorders.

                              

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Literature review current through: Oct 2014. | This topic last updated: Nov 26, 2013.
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References
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  1. Saxon A, Kobayashi RH, Stevens RH, et al. In vitro analysis of humoral immunity in antibody deficiency with normal immunoglobulins. Clin Immunol Immunopathol 1980; 17:235.
  2. French MA, Harrison G. Systemic antibody deficiency in patients without serum immunoglobulin deficiency or with selective IgA deficiency. Clin Exp Immunol 1984; 56:18.
  3. Ambrosino DM, Siber GR, Chilmonczyk BA, et al. An immunodeficiency characterized by impaired antibody responses to polysaccharides. N Engl J Med 1987; 316:790.
  4. Ambrosino DM, Umetsu DT, Siber GR, et al. Selective defect in the antibody response to Haemophilus influenzae type b in children with recurrent infections and normal serum IgG subclass levels. J Allergy Clin Immunol 1988; 81:1175.
  5. Sorensen RU, Leiva LE, Giangrosso PA, et al. Response to a heptavalent conjugate Streptococcus pneumoniae vaccine in children with recurrent infections who are unresponsive to the polysaccharide vaccine. Pediatr Infect Dis J 1998; 17:685.
  6. Sorensen RU, Leiva LE, Javier FC 3rd, et al. Influence of age on the response to Streptococcus pneumoniae vaccine in patients with recurrent infections and normal immunoglobulin concentrations. J Allergy Clin Immunol 1998; 102:215.
  7. Gross S, Blaiss MS, Herrod HG. Role of immunoglobulin subclasses and specific antibody determinations in the evaluation of recurrent infection in children. J Pediatr 1992; 121:516.
  8. Sanders LA, Rijkers GT, Kuis W, et al. Defective antipneumococcal polysaccharide antibody response in children with recurrent respiratory tract infections. J Allergy Clin Immunol 1993; 91:110.
  9. Zora JA, Silk HJ, Tinkelman DG. Evaluation of postimmunization pneumococcal titers in children with recurrent infections and normal levels of immunoglobulin. Ann Allergy 1993; 70:283.
  10. Knutsen AP. Patients with Igg subclass and/or selective antibody deficiency to polysaccharide antigens: initiation of a controlled clinical trial of intravenous immune globulin. J Allergy Clin Immunol 1989; 84:640.
  11. Herer B, Labrousse F, Mordelet-Dambrine M, et al. Selective IgG subclass deficiencies and antibody responses to pneumococcal capsular polysaccharide antigen in adult community-acquired pneumonia. Am Rev Respir Dis 1990; 142:854.
  12. Bernatowska-Matuszkiewicz E, Pac M, Pum M, et al. IgG subclasses and antibody response to pneumococcal capsular polysaccharides in children with severe sinopulmonary infections and asthma. Immunol Invest 1991; 20:173.
  13. Rijkers GT, Sanders LA, Zegers BJ. Anti-capsular polysaccharide antibody deficiency states. Immunodeficiency 1993; 5:1.
  14. Wood PM, Mayne A, Joyce H, et al. A mutation in Bruton's tyrosine kinase as a cause of selective anti-polysaccharide antibody deficiency. J Pediatr 2001; 139:148.
  15. Sorensen RU, Moore C. Antibody deficiency syndromes. Pediatr Clin North Am 2000; 47:1225.
  16. Barrett DJ, Ayoub EM. IgG2 subclass restriction of antibody to pneumococcal polysaccharides. Clin Exp Immunol 1986; 63:127.
  17. Lortan JE, Kaniuk AS, Monteil MA. Relationship of in vitro phagocytosis of serotype 14 Streptococcus pneumoniae to specific class and IgG subclass antibody levels in healthy adults. Clin Exp Immunol 1993; 91:54.
  18. Lim PL, Lau YL. Occurrence of IgG subclass antibodies to ovalbumin, avidin, and pneumococcal polysaccharide in children. Int Arch Allergy Immunol 1994; 104:137.
  19. Siber GR, Schur PH, Aisenberg AC, et al. Correlation between serum IgG-2 concentrations and the antibody response to bacterial polysaccharide antigens. N Engl J Med 1980; 303:178.
  20. Sorensen RU, Hidalgo H, Moore C, Leiva LE. Post-immunization pneumococcal antibody titers and IgG subclasses. Pediatr Pulmonol 1996; 22:167.
  21. Sanders LA, Rijkers GT, Tenbergen-Meekes AM, et al. Immunoglobulin isotype-specific antibody responses to pneumococcal polysaccharide vaccine in patients with recurrent bacterial respiratory tract infections. Pediatr Res 1995; 37:812.
  22. Oxelius VA, Berkel AI, Hanson LA. IgG2 deficiency in ataxia-telangiectasia. N Engl J Med 1982; 306:515.
  23. Kalfa VC, Roberts RL, Stiehm ER. The syndrome of chronic mucocutaneous candidiasis with selective antibody deficiency. Ann Allergy Asthma Immunol 2003; 90:259.
  24. Sorensen RU, Moore C. Immunology in the pediatrician's office. Pediatr Clin North Am 1994; 41:691.
  25. Hidalgo H, Moore C, Leiva LE, Sorensen RU. Preimmunization and postimmunization pneumococcal antibody titers in children with recurrent infections. Ann Allergy Asthma Immunol 1996; 76:341.
  26. Epstein MM, Gruskay F. Selective deficiency in pneumococcal antibody response in children with recurrent infections. Ann Allergy Asthma Immunol 1995; 75:125.
  27. Ekdahl K, Braconier JH, Svanborg C. Immunoglobulin deficiencies and impaired immune response to polysaccharide antigens in adult patients with recurrent community-acquired pneumonia. Scand J Infect Dis 1997; 29:401.
  28. Javier FC 3rd, Moore CM, Sorensen RU. Distribution of primary immunodeficiency diseases diagnosed in a pediatric tertiary hospital. Ann Allergy Asthma Immunol 2000; 84:25.
  29. van Kessel DA, van Velzen-Blad H, van den Bosch JM, Rijkers GT. Impaired pneumococcal antibody response in bronchiectasis of unknown aetiology. Eur Respir J 2005; 25:482.
  30. Orange JS, Ballow M, Stiehm ER, et al. Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol 2012; 130:S1.
  31. MacDonald NE, Halperin SA, Law BJ, et al. Induction of immunologic memory by conjugated vs plain meningococcal C polysaccharide vaccine in toddlers: a randomized controlled trial. JAMA 1998; 280:1685.
  32. Go ES, Ballas ZK. Anti-pneumococcal antibody response in normal subjects: a meta-analysis. J Allergy Clin Immunol 1996; 98:205.
  33. Centers for Disease Control and Prevention (CDC). Recommended childhood immunization schedule--United States, 2002. MMWR Morb Mortal Wkly Rep 2002; 51:31.
  34. Zielen S, Bühring I, Strnad N, et al. Immunogenicity and tolerance of a 7-valent pneumococcal conjugate vaccine in nonresponders to the 23-valent pneumococcal vaccine. Infect Immun 2000; 68:1435.
  35. Gibson A, Edgar JD, Neville CE, et al. Effect of fruit and vegetable consumption on immune function in older people: a randomized controlled trial. Am J Clin Nutr 2012; 96:1429.
  36. Sorensen RU, et al, unpublished observations.
  37. Wasserman RL, Sorensen RU. Evaluating children with respiratory tract infections: the role of immunization with bacterial polysaccharide vaccine. Pediatr Infect Dis J 1999; 18:157.
  38. Centers for Disease Control and Prevention (CDC). Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine for adults with immunocompromising conditions: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rep 2012; 61:816.
  39. Shrimpton A, Duddridge M, Ziegler-Heitbrock L. Vaccination with polysaccharide-conjugate-vaccines in adult patients with specific antibody deficiency. Vaccine 2006; 24:3574.
  40. Wolpert J, Knutsen AP. Natural history of selective antibody deficiency to bacterial polysaccharide antigens in children. Pediatr Asthma Allergy Immunol 1998; 12:183.
  41. Cohn JA, Skorpinski E, Cohn JR. Prevention of pneumococcal infection in a patient with normal immunoglobulin levels but impaired polysaccharide antibody production. Ann Allergy Asthma Immunol 2006; 97:603.
  42. Shearer WT, Buckley RH, Engler RJ, et al. Practice parameters for the diagnosis and management of immunodeficiency. The Clinical and Laboratory Immunology Committee of the American Academy of Allergy, Asthma, and Immunology (CLIC-AAAAI). Ann Allergy Asthma Immunol 1996; 76:282.
  43. Ortigas AP, Leiva LE, Moore C, et al. Natural history of specific antibody deficiency after IgG replacement therapy. Ann Allergy Asthma Immunol 1999; 82:71.
  44. Alachkar H, Taubenheim N, Haeney MR, et al. Memory switched B cell percentage and not serum immunoglobulin concentration is associated with clinical complications in children and adults with specific antibody deficiency and common variable immunodeficiency. Clin Immunol 2006; 120:310.