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    Primary Immune Deficiency

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    This document provides guidelines for referring patients with suspected primary immune deficiency. It lists several warning signs that warrant referral to an allergist or immunologist for diagnosis and management. These include frequent or unusual infections, failure to gain weight or grow normally, and a family history of immune deficiency. Advanced diagnostic testing by specialists is important to properly diagnose and treat primary immunodeficiencies. Immunoglobulin therapy and other immunologic treatments can improve immunity, reduce infections, improve organ function, and prevent complications, leading to an improved quality of life and possibly curing some deficiencies.

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    Primary Immune Deficiency

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    razedinblack2
    59 views3 pages
    This document provides guidelines for referring patients with suspected primary immune deficiency. It lists several warning signs that warrant referral to an allergist or immunologist for diagnosis and management. These include frequent or unusual infections, failure to gain weight or grow normally, and a family history of immune deficiency. Advanced diagnostic testing by specialists is important to properly diagnose and treat primary immunodeficiencies. Immunoglobulin therapy and other immunologic treatments can improve immunity, reduce infections, improve organ function, and prevent complications, leading to an improved quality of life and possibly curing some deficiencies.

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    This document provides guidelines for referring patients with suspected primary immune deficiency. It lists several warning signs that warrant referral to an allergist or immunologist for diagnosis and management. These include frequent or unusual infections, failure to gain weight or grow normally, and a family history of immune deficiency. Advanced diagnostic testing by specialists is important to properly diagnose and treat primary immunodeficiencies. Immunoglobulin therapy and other immunologic treatments can improve immunity, reduce infections, improve organ function, and prevent complications, leading to an improved quality of life and possibly curing some deficiencies.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PDF, TXT or read online from Scribd
    Download as pdf or txt
    59 views3 pages

    Primary Immune Deficiency

    Uploaded by

    razedinblack2
    This document provides guidelines for referring patients with suspected primary immune deficiency. It lists several warning signs that warrant referral to an allergist or immunologist for diagnosis and management. These include frequent or unusual infections, failure to gain weight or grow normally, and a family history of immune deficiency. Advanced diagnostic testing by specialists is important to properly diagnose and treat primary immunodeficiencies. Immunoglobulin therapy and other immunologic treatments can improve immunity, reduce infections, improve organ function, and prevent complications, leading to an improved quality of life and possibly curing some deficiencies.

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    Attribution Non-Commercial (BY-NC)
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    of 3

    Table 12.

    Primary Immune Deficiency


    Updates are in red type

    Referral Guideline Rationale Evidence Type


    Any of the following warning signs: Frequent infection, unusual infections or unusual complications of usual Diagnostic
    1-7
    infections are the most frequent presentation of immune deficiency .
    • Eight or more new infections within one year; Advanced diagnostic strategies are necessary to ensure appropriate Indirect outcome
    1,6-9
    • Two or more serious sinus infections within one year; diagnosis and treatment. Allergist/immunologists are trained to (immunologic therapy)
    10
    • Two or more months on antibiotic with little or no effect; diagnose and treat primary immunodeficiency . Immunologic therapy
    11,12 13-15 16
    • Two or more pneumonias within 1 year; improves immunity , reduces infections
    1
    , improves organ function ,
    17
    • Failure of an infant to gain weight or grow normally; prevents complications , improves quality of life , and may be
    18,19
    • Recurrent deep skin or organ abscesses; curative in patients with primary immune deficiencies.
    • Persistent thrush in mouth or elsewhere on skin after age
    1 year;
    • Need for intravenous antibiotics to clear infections;
    • Two or more deep seated infections;
    • A family history of immune deficiency.

    References:

    1. Bonilla, F.A., Bernstein, L.I., Khan, D.A., Balas, Z.K., Chinen, J., Fank, M.M., Kobrynski, L.J., Levinson, A.I., Mazer, B., Nelson, R.P.,
    Orange, J.S., Routes, J., Shearer, W.T., Sorensen, R.U. Practice Parameter for the diagnosis and management of primary
    immunodeficiency. 2005, Ann. Allergy. 94:S1-62. Evidence grade: IV

    2. Ballow M. Primary immunodeficiency disorders: antibody deficiency. J Allergy Clin Immunol. 2002; 109:581-91. Evidence grade: IV

    3. Buckley RH. Primary cellular immunodeficiencies. J Allergy Clin Immunol. 2002; 109:747-57. Evidence grade: IV

    4. Primary immunodeficiency diseases. Report of an IUIS Scientific Committee. International Union of Immunological Societies. Clin Exp
    Immunol. 1999; 118 Suppl 1:1-28. Evidence grade: IV

    5. Arnaiz-Villena A, Rodriguez-Gallego C, Timon M et al. Diseases involving the T-cell receptor/Cd3 complex. Crit Rev Oncol Hematol. 1995;
    19:131-47. Evidence grade: IV
    6. Champi C. Primary immunodeficiency disorders in children: prompt diagnosis can lead to lifesaving treatment. J Pediatr Health Care.
    2002; 16:16-21. Evidence grade: IV

    7. Fleisher TA. Evaluation of the potentially immunodeficient patient. Adv Intern Med. 1996; 41:1-30. Evidence grade: IV

    8. Wasserman, R. L., and R. U. Sorensen. 1999. Evaluating children with respiratory tract infections: the role of immunization with bacterial
    polysaccharide vaccine. Pediatr Infect Dis J 18:157. Evidence grade: III

    9. Geha RS, Notarangelo LD, Casanova JL, et al. International Union of Immunological Societies Primary Immunodeficiency Diseases
    Classification Committee. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary
    Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol. 2007;120(4):776-94. Evidence grade: IV

    10. Allergy and Immunology Core Curriculum Outline 1996. Core Curriculum Subcommittee of the Training Program Directors. American
    Academy of Allergy, Asthma and Immunology. J Allergy Clin Immunol 1996;98(6pt.1):1012-5, updated in 2002
    (http://www.aaaai.org/professionals/careers/training_programs.stm). Evidence grade: IV

    11. Hershfield, M. S. 1995. PEG-ADA replacement therapy for adenosine deaminase deficiency: an update after 8.5 years. Clin Immunol
    Immunopathol 76:S228. Evidence grade: III

    12. Cunningham-Rundles, C., C. Bodian, H. D. Ochs, S. Martin, M. Reiter-Wong, and Z. Zhuo. 2001. Long-term low-dose IL-2 enhances
    immune function in common variable immunodeficiency. Clin Immunol 100:181. Evidence grade: II

    13. Nydahl-Persson, K., A. Petterson, and A. Fasth. 1995. A prospective, double-blind, placebo-controlled trial of i.v. immunoglobulin and
    trimethoprim-sulfamethoxazole in children with recurrent respiratory tract infections. Acta Paediatr 84:1007. Evidence grade: Ib

    14. Gallin, J. I., D. W. Alling, H. L. Malech, R. Wesley, D. Koziol, B. Marciano, E. M. Eisenstein, M. L. Turner, E. S. DeCarlo, J. M. Starling,
    and S. M. Holland. 2003. Itraconazole to prevent fungal infections in chronic granulomatous disease. N Engl J Med 348:2416. Evidence
    grade: Ib

    15. Busse, P. J., S. Razvi, and C. Cunningham-Rundles. 2002. Efficacy of intravenous immunoglobulin in the prevention of pneumonia in
    patients with common variable immunodeficiency. J Allergy Clin Immunol 109:1001. Evidence grade: III

    16. de Gracia, J., M. Vendrell, A. Alvarez, E. Pallisa, M. J. Rodrigo, D. de la Rosa, F. Mata, J. Andreu, and F. Morell. 2004. Immunoglobulin
    therapy to control lung damage in patients with common variable immunodeficiency. Int Immunopharmacol 4:745. Evidence grade: II
    17. Gardulf, A., U. Nicolay, D. Math, O. Asensio, E. Bernatowska, A. Bock, B. T. Costa-Carvalho, C. Granert, S. Haag, D. Hernandez, P.
    Kiessling, J. Kus, N. Matamoros, T. Niehues, S. Schmidt, I. Schulze, and M. Borte. 2004. Children and adults with primary antibody
    deficiencies gain quality of life by subcutaneous IgG self-infusions at home. J Allergy Clin Immunol 114:936. Evidence grade: II

    18. Horwitz, M. E., A. J. Barrett, M. R. Brown, C. S. Carter, R. Childs, J. I. Gallin, S. M. Holland, G. F. Linton, J. A. Miller, S. F. Leitman, E. J.
    Read, and H. L. Malech. 2001. Treatment of chronic granulomatous disease with nonmyeloablative conditioning and a T-cell-depleted
    hematopoietic allograft. N Engl J Med 344:881. Evidence grade: II

    19. Buckley, R. H., S. E. Schiff, R. I. Schiff, L. Markert, L. W. Williams, J. L. Roberts, L. A. Myers, and F. E. Ward. 1999. Hematopoietic stem-
    cell transplantation for the treatment of severe combined immunodeficiency. N Engl J Med 340:508. Evidence grade: III

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