Practice parameter Practice parameter for the diagnosis and management of primary immunodeficiency Francisco A. Bonilla, MD, PhD, David A. Khan, MD, Zuhair K. Ballas, MD, Javier Chinen, MD, PhD, Michael M. Frank, MD, Joyce T. Hsu, MD, Michael Keller, MD, Lisa J. Kobrynski, MD, Hirsh D. Komarow, MD, Bruce Mazer, MD, Robert P. Nelson, Jr, MD, Jordan S. Orange, MD, PhD, John M. Routes, MD, William T. Shearer, MD, PhD, Ricardo U. Sorensen, MD, James W. Verbsky, MD, PhD, David I. Bernstein, MD, Joann Blessing-Moore, MD, David Lang, MD, Richard A. Nicklas, MD, John Oppenheimer, MD, Jay M. Portnoy, MD, Christopher R. Randolph, MD, Diane Schuller, MD, Sheldon L. Spector, MD, Stephen Tilles, MD, Dana Wallace, MD Chief Editor: Francisco A. Bonilla, MD, PhD Co-Editor: David A. Khan, MD Members of the Joint Task Force on Practice Parameters: David I. Bernstein, MD, Joann Blessing-Moore, MD, David Khan, MD, David Lang, MD, Richard A. Nicklas, MD, John Oppenheimer, MD, Jay M. Portnoy, MD, Christopher R. Randolph, MD, Diane Schuller, MD, Sheldon L. Spector, MD, Stephen Tilles, MD, Dana Wallace, MD Primary Immunodeficiency Workgroup: Chairman: Francisco A. Bonilla, MD, PhD Members: Zuhair K. Ballas, MD, Javier Chinen, MD, PhD, Michael M. Frank, MD, Joyce T. Hsu, MD, Michael Keller, MD, Lisa J. Kobrynski, MD, Hirsh D. Komarow, MD, Bruce Mazer, MD, Robert P. Nelson, Jr, MD, Jordan S. Orange, MD, PhD, John M. Routes, MD, William T. Shearer, MD, PhD, Ricardo U. Sorensen, MD, James W. Verbsky, MD, PhD GlaxoSmithKline, Merck, and Aerocrine; has received payment for lectures from Genentech/ These parameters were developed by the Joint Task Force on Practice Parameters, representing Novartis, GlaxoSmithKline, and Merck; and has received research support from Genentech/ the American Academy of Allergy, Asthma & Immunology; the American College of Novartis and Merck. R. A. Nicklas is a committee chair for the American College of Allergy, Allergy, Asthma & Immunology; and the Joint Council of Allergy, Asthma & Immunology. Asthma & Immunology. J. Oppenheimer has consultant arrangements with AstraZeneca, H.D.K. is supported by the Division of Intramural Research, National Institute of Allergy and GlaxoSmithKline, Sunovion, Mylan, and Sanofi; has received research support from Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, Md. AstraZeneca, GlaxoSmithKline, Merck, Novartis, Boehringer Ingelheim, and MedImmune; Disclosure of potential conflict of interest: F. A. Bonilla has consultant arrangements with has provided legal consultation or expert witness testimony in cases related to malpractice; is ADMA Biologics, Baxter, Cowen Group, CSL Behring, the Gerson-Lehrman Group, Grand chairman of the American Board of Allergy and Immunology; and is Associate Editor of the Rounds Health, and the Immune Deficiency Foundation; has received research support from Annals of Allergy. J. M. Portnoy has received payment for lectures from Thermo Fisher and CSL Behring; has received payment for lectures from Albany Medical College; has received Mylan and has consultant arrangements with Thermo Fisher and Sanofi. C. R. Randolph has royalties from UpToDate in Medicine; and has received travel support from CSL Behring. D. received payment for lectures from GlaxoSmithKline, TEVA, ViroPharma, Merck, and Dey; A. Khan has received payment for lectures from Genentech, Merck, Baxter, and Viropharma; has received research support from GlaxoSmithKline, Merck, Amgen, and Genentech/Novar- has received research support from the VanbergFamily Foundation and the National Institutes tis; and has consultant arrangements with AstraZeneca and Meda. D. Schuller has received of Health (NIH)/National Institute of Mental Health; is the Allied Health Chair for the travel support from the Joint Council of Allergy, Asthma & Immunology for Joint Task Force American College of Allergy, Asthma & Immunology; and is a member of the Joint Task meetings. S. L. Spector has stock in GlaxoSmithKline and Merck; has consultant arrange- Force to Practice Parameters for the Joint Council on Allergy, Asthma & Immunology. Z. K. ments with Hycor; has received research support from AstraZeneca, GlaxoSmithKline, Am- Ballas has consulting arrangements with the Immune Deficiency Foundation; has received gen, Genentech, Novartis, Teva, Mylan, Sanofi, and Boehringer Ingelheim; and is a speaker/ research support from the NIH Cancer Center; and has received royalties from UpToDate. moderator for the American College of Allergy, Asthma & Immunology. S. Tilles has consul- M. M. Frank is on the DCMB for Biocryst and has received travel support from CSL Behring. tant arrangements with SRXA, Sunovion, and Hycor; has received research support from As- M. Keller has received research support from the Jeffrey Modell Foundation. L. J. Kobrynski tellas, Amphastar, Medimmune, Cephalon, Genentech, Merck, TEVA, Sunovion, Boehringer has consultant arrangements with CSL Behring; has received research support from the Ingelheim, Nutricia, Array, Rigel, and AstraZeneca; is Associate Editor of AllergyWatch and Centers for Disease Control and Prevention Foundation through the NIH; has received the Annals of Allergy; is Assistant Editor of the Joint Task Force for Practice Parameters; and payment for lectures from Baxter Healthcare; and has received travel support and speakers’ is on the Executive Committee for the Seattle Food Allergy Consortium. D. Wallace has fees from the Immune Deficiency Foundation. J. S. Orange has consultant arrangements with received payment for lectures from TEVA,Mylan Labs, and the American College of Allergy, Baxter Healthcare, CSL Behring, ASD Healthcare, ADMA Biologics, and Walgreens; has Asthma & Immunology; is an advisor for Sanofi and Sunovion; is on the Executive Commit- received research support from CSL Behring; has received payment for lectures from Baxter tee of the American College of Allergy, Asthma & Immunology; and is on the Board of Di- Healthcare; has received royalties from UpToDate, UniMed, and Springer; and is on the rectors for the World Allergy Organization. The rest of the authors declare that they have no Medical Advisory Council of the Immune Deficiency Foundation. W. T. Shearer is employed relevant conflicts of interest. by Baylor College of Medicine. J. W. Verbsky has received royalties from UpToDate. D. I. Corresponding author: Francisco A. Bonilla, MD, PhD, Boston Children’s Hospital, Boston, Bernstein has received research support from TEVA, Genentech, Pfizer, Merck, Meda, MA 02115. E-mail: [email protected]. GlaxoSmithKline, Array, Cephalon, and MedImmune and has provided legal consultation or expert witness testimony in cases related to anaphylaxis, contact dermatitis, and occupational Received for publication December 30, 2014; revised April 18, 2015; accepted for publication April 23, 2015. asthma. J. Blessing-Moore has received payment for lectures from Meda, Alcon, TEVA, Available online September 12, 2015. Sunovion, Genentech/Novartis, Merck, and AstraZeneca; has received research support from 0091-6749 Meda; and serves on committees for the American College of Chest Physicians, the American http://dx.doi.org/10.1016/j.jaci.2015.04.049 College of Allergy, Asthma & Immunology, the American Academy of Allergy, Asthma & Immunology, and the American Thoracic Society. D. Lang has consultant arrangements with 1186 J ALLERGY CLIN IMMUNOL BONILLA ET AL 1187 VOLUME 136, NUMBER 5 The American Academy of Allergy, Asthma & Immunology David M. Lang, MD (AAAAI) and the American College of Allergy, Asthma & Head, Allergy/Immunology Section Immunology (ACAAI) have jointly accepted responsibility for Respiratory Institute establishing the ‘‘Practice parameter for the diagnosis and Director, Allergy and Immunology Fellowship Training management of primary immunodeficiency.’’ This is a complete Program and comprehensive document at the current time. The medical Cleveland Clinic Foundation environment is a changing environment, and not all Cleveland, Ohio recommendations will be appropriate for all patients. Because this document incorporated the efforts of many participants, no Richard A. Nicklas, MD single individual, including those who served on the Joint Task Clinical Professor of Medicine Force, is authorized to provide an official AAAAI or ACAAI George Washington Medical Center interpretation of these practice parameters. Any request for Washington, DC 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 John Oppenheimer, MD Council of Allergy, Asthma & Immunology. These parameters Department of Internal Medicine are not designed for use by pharmaceutical companies in drug New Jersey Medical School promotion. (J Allergy Clin Immunol 2015;136:1186-205.) Pulmonary and Allergy Associates Morristown, NJ Discuss this article on the JACI Journal Club blog: www.jaci- online.blogspot.com. Jay M. Portnoy, MD Previously published practice parameters of the Joint Chief, Section of Allergy, Asthma & Immunology Task Force on Practice Parameters for Allergy & Immunology The Children’s Mercy Hospital are available at http://www.JCAAI.org or http://www.allergy Professor of Pediatrics parameters.org. University of Missouri-Kansas City School of Medicine Kansas City, Mo CONTRIBUTORS The Joint Task Force has made a concerted effort to acknowl- Christopher C. Randolph, MD edge all contributors to this parameter. If any contributors Professor have been excluded