TY - JOUR
T1 - BCG vaccination in patients with severe combined immunodeficiency
T2 - Complications, risks, and vaccination policies
AU - Marciano, Beatriz E.
AU - Huang, Chiung Yu
AU - Joshi, Gyan
AU - Rezaei, Nima
AU - Carvalho, Beatriz Costa
AU - Allwood, Zoe
AU - Ikinciogullari, Aydan
AU - Reda, Shereen M.
AU - Gennery, Andrew
AU - Thon, Vojtech
AU - Espinosa-Rosales, Francisco
AU - Al-Herz, Waleed
AU - Porras, Oscar
AU - Shcherbina, Anna
AU - Szaflarska, Anna
AU - Kiliç, Şebnem
AU - Franco, Jose L.
AU - Gómez Raccio, Andrea C.
AU - Roxo, Persio
AU - Esteves, Isabel
AU - Galal, Nermeen
AU - Grumach, Anete Sevciovic
AU - Al-Tamemi, Salem
AU - Yildiran, Alisan
AU - Orellana, Julio C.
AU - Yamada, Masafumi
AU - Morio, Tomohiro
AU - Liberatore, Diana
AU - Ohtsuka, Yoshitoshi
AU - Lau, Yu Lung
AU - Nishikomori, Ryuta
AU - Torres-Lozano, Carlos
AU - Mazzucchelli, Juliana T.L.
AU - Vilela, Maria M.S.
AU - Tavares, Fabiola S.
AU - Cunha, Luciana
AU - Pinto, Jorge A.
AU - Espinosa-Padilla, Sara E.
AU - Hernandez-Nieto, Leticia
AU - Elfeky, Reem A.
AU - Ariga, Tadashi
AU - Toshio, Heike
AU - Dogu, Figen
AU - Cipe, Funda
AU - Formankova, Renata
AU - Nuñez-Nuñez, M. Enriqueta
AU - Bezrodnik, Liliana
AU - Marques, Jose Gonçalo
AU - Pereira, María I.
AU - Listello, Viviana
AU - Slatter, Mary A.
AU - Nademi, Zohreh
AU - Kowalczyk, Danuta
AU - Fleisher, Thomas A.
AU - Davies, Graham
AU - Neven, Bénédicte
AU - Rosenzweig, Sergio D.
N1 - Funding Information:
Supported by the Intramural Research Program of the National Institutes of Health, National Institute of Allergy and Infectious Disease . V.T. was supported by the European Community’s Seventh Framework Program FP7/2007-2013 under grant agreement no. 201549 (EURO-PADnet HEALTH-F2-2008-201549).
Funding Information:
Disclosure of potential conflict of interest: N. Rezaei is employed by and has received research support from Tehran University Med Sci , has received royalties from Springer , and has been supported by an American Academy of Allergy, Asthma & Immunology (AAAAI) Young Investigator Award . B. Costa Carvalho has received payment for development of educational presentations from the Federal University of Sao Paulo (FAPESP) and has received travel support from Octapharma. V. Thon has received research support from EURO-PADnet HEALTH ( F2-2008-201549 ). J. L. Franco has received consultancy fees from Baxter and Kedrion and has received lecture fees from Grifols . A. Sevciovic Grumach is a board member for Latin American Society for Immunodeficiencies and has received consultancy fees and lecture fees from SHIRE and CSL . T. Morio has received grant-in-aids for scientific research from the Japan Science and Technology Agency ; the Ministry of Education, Culture, and Sport ; and the Ministry of Health, Labour, and Welfare in Japan and has received lecture fees from Abbvie , CSL Behring , Chugai Pharmaceutical , Meiji Pharmaceuticals , Teijin Pharma , and Toray Medical . S. D. Rosenzweig has received consultancy fees from InPractice and UpToDate . The rest of the authors declare that they have no relevant conflicts of interest.
PY - 2014/4
Y1 - 2014/4
N2 - Background Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected. Objectives We sought to describe the complications and risks associated with BCG vaccination in patients with SCID. Methods An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed. Results Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (≥1 month) showed an increased prevalence of complications (P =.006) and death caused by BCG-associated complications (P <.0001). The odds of experiencing complications among patients with T-cell numbers of 250/μL or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P =.001) than among those with T-cell numbers of greater than 250/μL. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160 patients treated with antimycobacterial therapy for a symptomatic BCG infection (P<.0001). Conclusions BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.
AB - Background Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected. Objectives We sought to describe the complications and risks associated with BCG vaccination in patients with SCID. Methods An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed. Results Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (≥1 month) showed an increased prevalence of complications (P =.006) and death caused by BCG-associated complications (P <.0001). The odds of experiencing complications among patients with T-cell numbers of 250/μL or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P =.001) than among those with T-cell numbers of greater than 250/μL. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160 patients treated with antimycobacterial therapy for a symptomatic BCG infection (P<.0001). Conclusions BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.
KW - BCG
KW - Primary immunodeficiency
KW - hematopoietic stem cell transplant
KW - immune reconstitution syndrome
KW - mycobacteria
KW - newborn screening
KW - severe combined immunodeficiency
KW - vaccine
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U2 - 10.1016/j.jaci.2014.02.028
DO - 10.1016/j.jaci.2014.02.028
M3 - Article
C2 - 24679470
AN - SCOPUS:84897136937
VL - 133
SP - 1134
EP - 1141
JO - Journal of Allergy and Clinical Immunology
JF - Journal of Allergy and Clinical Immunology
SN - 0091-6749
IS - 4
ER -