Reviews and feature article
Practice parameter for the diagnosis and management of primary immunodeficiency

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The American Academy of Allergy, Asthma & Immunology (AAAAI) and the American College of Allergy, Asthma & Immunology (ACAAI) have jointly accepted responsibility for establishing the “Practice parameter for the diagnosis and management of primary immunodeficiency.” This is a complete and comprehensive document at the current time. The medical environment is a changing environment, and not all recommendations will be appropriate for all patients. Because this document incorporated the efforts of many participants, no single individual, including those who served on the Joint Task Force, is authorized to provide an official AAAAI or ACAAI interpretation of these practice parameters. Any request for information about or an interpretation of these practice parameters by the AAAAI or ACAAI should be directed to the Executive Offices of the AAAAI, the ACAAI, and the Joint Council of Allergy, Asthma & Immunology. These parameters are not designed for use by pharmaceutical companies in drug promotion.

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Contributors

The Joint Task Force has made a concerted effort to acknowledge all contributors to this parameter. If any contributors have been excluded inadvertently, the Task Force will ensure that appropriate recognition of such contributions is made subsequently.

Workgroup Chair and Chief Editor

Francisco A. Bonilla, MD, PhD (Chair)

Senior Associate Physician, Boston Children's Hospital

Associate Professor of Pediatrics, Harvard Medical School

Boston, Mass

Joint Task Force Liaison and Co-Editor

David A. Khan, MD

Associate Professor of Internal Medicine

University of Texas Southwestern Medical Center

Dallas, Tex

Joint Task Force Members

David I. Bernstein, MD

Professor of Clinical Medicine and Environmental Health

Division of Immunology, Allergy and Rheumatology

University of Cincinnati College of Medicine

Cincinnati, Ohio

Joann Blessing-Moore, MD

Adjunct Professor of Medicine and Pediatrics

Stanford University Medical Center

Department of Immunology

Palo Alto, Calif

David M. Lang, MD

Head, Allergy/Immunology Section

Respiratory Institute

Director, Allergy and Immunology Fellowship Training Program

Cleveland Clinic Foundation

Cleveland, Ohio

Workgroup Members

Zuhair K. Ballas, MD

Director, Immunology Division

Department of Internal Medicine, University of Iowa and the Iowa City Veteran's

Administration Medical Center

Iowa City, Iowa

Javier Chinen, MD, PhD

Allergy and Immunology Consultant

Lake Houston Allergy and Immunology

Humble, Tex

Michael M. Frank, MD

Samuel L. Katz Professor and Chairman of Pediatrics

Professor of Immunology and Medicine, Department of Pediatrics, Children's Health Center

Duke University Medical Center

Durham, NC

Joyce T. Hsu, MD

Division of

Reviewers

Mark Ballow, MD, St Petersburg, Fla

Thomas A. Fleisher, MD, Bethesda, Md

Maite de la Morena, MD, Dallas, Tex

Elena Perez, MD, Miami, Fla

Classification of recommendations and evidence

Classification of recommendations and evidence are listed in Table I.

Summary of conflict of interest disclosures

The following is a summary of interests disclosed on workgroup members' conflict of interest disclosure statements (not including information concerning family member interests). Completed conflict of interest disclosure statements are available on request.

Workgroup memberDisclosures
Francisco A. Bonilla, MD, PhDConsultant: ADMA Biologics; Baxter; The Cowen Group; CSL Behring; Gerson-Lehrman Group; Grand Rounds Health; Immune Deficiency Foundation. DSMB: Octapharma. UpToDate in Medicine.
David A.

Resolution of nondisqualifying interests

The Joint Task Force recognizes that experts in a field are likely to have interests that could come into conflict with the development of a completely unbiased and objective practice parameter. To take advantage of that expertise, a process has been developed to prevent potential conflicts from influencing the final document in a negative way.

At the workgroup level, members who have a potential conflict of interest either do not participate in discussions concerning topics related to the

Protocol for finding evidence

A search of the medical literature on PubMed was performed for a variety of terms that were considered relevant to this practice parameter. All reference types were included in the results. References identified as being relevant were searched for other relevant references. Published clinical studies were rated by category of evidence and used to establish the strength of the recommendations. The parameter was subsequently appraised by reviewers designated by the AAAAI and ACAAI. Based on this

Preface

The purpose of this “Practice parameter for the diagnosis and management of primary immunodeficiency” is to provide the consultant allergist/immunologist or other practitioner with a practical guide for the clinical recognition and diagnosis of immunodeficiency, along with the general principles that guide management of these disorders. This document was developed by a working group under the aegis of the 3 national allergy and immunology societies: the American Academy of Allergy, Asthma &

Executive summary

Primary immunodeficiencies are inherited disorders of immune system function that predispose affected subjects to an increased rate and severity of infection, immune dysregulation with autoimmune disease and aberrant inflammatory responses, and malignancy. Primary immunodeficiencies are distinct from secondary immunodeficiencies that occur, for example, during certain viral infections, after immunosuppression to prevent graft rejection after transplantation, during treatment of systemic

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These parameters were developed by the Joint Task Force on Practice Parameters, representing the American Academy of Allergy, Asthma & Immunology; the American College of Allergy, Asthma & Immunology; and the Joint Council of Allergy, Asthma & Immunology.

H.D.K. is supported by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, Md.

Disclosure of potential conflict of interest: F. A. Bonilla has consultant arrangements with ADMA Biologics, Baxter, Cowen Group, CSL Behring, the Gerson-Lehrman Group, Grand Rounds Health, and the Immune Deficiency Foundation; has received research support from CSL Behring; has received payment for lectures from Albany Medical College; has received royalties from UpToDate in Medicine; and has received travel support from CSL Behring. D. A. Khan has received payment for lectures from Genentech, Merck, Baxter, and Viropharma; has received research support from the Vanberg Family Foundation and the National Institutes of Health (NIH)/National Institute of Mental Health; is the Allied Health Chair for the American College of Allergy, Asthma & Immunology; and is a member of the Joint Task Force to Practice Parameters for the Joint Council on Allergy, Asthma & Immunology. Z. K. Ballas has consulting arrangements with the Immune Deficiency Foundation; has received research support from the NIH Cancer Center; and has received royalties from UpToDate. M. M. Frank is on the DCMB for Biocryst and has received travel support from CSL Behring. M. Keller has received research support from the Jeffrey Modell Foundation. L. J. Kobrynski has consultant arrangements with CSL Behring; has received research support from the Centers for Disease Control and Prevention Foundation through the NIH; has received payment for lectures from Baxter Healthcare; and has received travel support and speakers' fees from the Immune Deficiency Foundation. J. S. Orange has consultant arrangements with Baxter Healthcare, CSL Behring, ASD Healthcare, ADMA Biologics, and Walgreens; has received research support from CSL Behring; has received payment for lectures from Baxter Healthcare; has received royalties from UpToDate, UniMed, and Springer; and is on the Medical Advisory Council of the Immune Deficiency Foundation. W. T. Shearer is employed by Baylor College of Medicine. J. W. Verbsky has received royalties from UpToDate. D. I. Bernstein has received research support from TEVA, Genentech, Pfizer, Merck, Meda, GlaxoSmithKline, Array, Cephalon, and MedImmune and has provided legal consultation or expert witness testimony in cases related to anaphylaxis, contact dermatitis, and occupational asthma. J. Blessing-Moore has received payment for lectures from Meda, Alcon, TEVA, Sunovion, Genentech/Novartis, Merck, and AstraZeneca; has received research support from Meda; and serves on committees for the American College of Chest Physicians, the American College of Allergy, Asthma & Immunology, the American Academy of Allergy, Asthma & Immunology, and the American Thoracic Society. D. Lang has consultant arrangements with GlaxoSmithKline, Merck, and Aerocrine; has received payment for lectures from Genentech/Novartis, GlaxoSmithKline, and Merck; and has received research support from Genentech/Novartis and Merck. R. A. Nicklas is a committee chair for the American College of Allergy, Asthma & Immunology. J. Oppenheimer has consultant arrangements with AstraZeneca, GlaxoSmithKline, Sunovion, Mylan, and Sanofi; has received research support from AstraZeneca, GlaxoSmithKline, Merck, Novartis, Boehringer Ingelheim, and MedImmune; has provided legal consultation or expert witness testimony in cases related to malpractice; is chairman of the American Board of Allergy and Immunology; and is Associate Editor of the Annals of Allergy. J. M. Portnoy has received payment for lectures from Thermo Fisher and Mylan and has consultant arrangements with Thermo Fisher and Sanofi. C. R. Randolph has received payment for lectures from GlaxoSmithKline, TEVA, ViroPharma, Merck, and Dey; has received research support from GlaxoSmithKline, Merck, Amgen, and Genentech/Novartis; and has consultant arrangements with AstraZeneca and Meda. D. Schuller has received travel support from the Joint Council of Allergy, Asthma & Immunology for Joint Task Force meetings. S. L. Spector has stock in GlaxoSmithKline and Merck; has consultant arrangements with Hycor; has received research support from AstraZeneca, GlaxoSmithKline, Amgen, Genentech, Novartis, Teva, Mylan, Sanofi, and Boehringer Ingelheim; and is a speaker/moderator for the American College of Allergy, Asthma & Immunology. S. Tilles has consultant arrangements with SRXA, Sunovion, and Hycor; has received research support from Astellas, Amphastar, Medimmune, Cephalon, Genentech, Merck, TEVA, Sunovion, Boehringer Ingelheim, Nutricia, Array, Rigel, and AstraZeneca; is Associate Editor of AllergyWatch and the Annals of Allergy; is Assistant Editor of the Joint Task Force for Practice Parameters; and is on the Executive Committee for the Seattle Food Allergy Consortium. D. Wallace has received payment for lectures from TEVA, Mylan Labs, and the American College of Allergy, Asthma & Immunology; is an advisor for Sanofi and Sunovion; is on the Executive Committee of the American College of Allergy, Asthma & Immunology; and is on the Board of Directors for the World Allergy Organization. The rest of the authors declare that they have no relevant conflicts of interest.

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