Abstract
Purpose
Patients with primary immunodeficiency (PID) are at risk of serious complications. However, data on the incidence and causes of emergency hospital admissions are scarce. The primary objective of the present study was to describe emergency hospital admissions among patients with PID, with a view to identifying “at-risk” patient profiles.
Methods
We performed a prospective observational 12-month multicenter study in France via the CEREDIH network of regional PID reference centers from November 2010 to October 2011. All patients with PIDs requiring emergency hospital admission were included.
Results
A total of 200 admissions concerned 137 patients (73 adults and 64 children, 53% of whom had antibody deficiencies). Thirty admissions were reported for 16 hematopoietic stem cell transplantation recipients. When considering the 170 admissions of non-transplant patients, 149 (85%) were related to acute infections (respiratory tract infections and gastrointestinal tract infections in 72 (36%) and 34 (17%) of cases, respectively). Seventy-seven percent of the admissions occurred during winter or spring (December to May). The in-hospital mortality rate was 8.8% (12 patients); death was related to a severe infection in 11 cases (8%) and Epstein-Barr virus–induced lymphoma in 1 case. Patients with a central venous catheter (n = 19, 13.9%) were significantly more hospitalized for an infection (94.7%) than for a non-infectious reason (5.3%) (p = 0.04).
Conclusion
Our data showed that the annual incidence of emergency hospital admission among patients with PID is 3.4%. The leading cause of emergency hospital admission was an acute infection, and having a central venous catheter was associated with a significantly greater risk of admission for an infectious episode.
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23 June 2020
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Abbreviations
- CEREDIH:
-
Centre de Référence des Déficits Immunitaires Héréditaires
- CGD:
-
Chronic granulomatous disease
- CNS:
-
Central nervous system
- COPD:
-
Chronic obstructive pulmonary disease
- EBV:
-
Epstein-Barr virus
- HRQoL:
-
Health-related quality of life
- HSCT:
-
Hematopoietic stem cell transplantation
- HSV:
-
Herpes simplex virus
- NS:
-
Non-significant
- PID:
-
Primary immunodeficiency
- RSV:
-
Respiratory syncytial virus
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Acknowledgments
The European Society for Immunodeficiencies online database was used to collect the study data. The members of the CEREDIH French PID study group are (in alphabetical order) Laurent Aaron+, Daniel Adoue, Claire Aguilar, Nathalie Aladjidi, Alexandre Alcais, Zahir Amoura, Philippe Arlet, Corinne Armari-Alla, Brigitte Bader-Meunier, Sophie Bayart, Yves Bertrand, Boris Bienvenu, Stéphane Blanche, Damien Bodet, Bernard Bonnotte, Raphaël Borie, Patrick Boutard, Claire Briandet, Jean-Paul Brion, Jacques Brouard, Sarah Cohen-Beaussant, Laurence Costes, Louis-Jean Couderc, Pierre Cougoul, Virginie Courteille, Geneviève de Saint Basile, Catherine Devoldere, Anne Deville, Jean Donadieu, Eric Dore, Fabienne Dulieu, Christine Edan, Natacha Entz-Werle, Claire Fieschi, Amandine Forestier, Fanny Fouyssac, Vincent Gajdos, Lionel Galicier, Virginie Gandemer, Martine Gardembas, Catherine Gaud, Gaelle Guillerm, Eric Hachulla, Mohamed Hamidou, Olivier Hermine, Cyrille Hoarau, Sébastien Humbert, Arnaud Jaccard, Serge Jacquot, Jean-Philippe Jais, Roland Jaussaud, Pierre-Yves Jeandel, Kamila Kebaili, Anne-Sophie Korganow, Olivier Lambotte, Fanny Lanternier, Claire Larroche, Anne-Sophie Lascaux, Emmanuelle Le Moigne, Vincent Le Moing, Yvon Lebranchu, Marc Lecuit, Guillaume Lefevre, Richard Lemal, Valérie Li Thiao Te, Aude Marie-Cardine, Nicolas Martin Silva, Agathe Masseau, Christian Massot, Françoise Mazingue, Etienne Merlin, Gérard Michel, Frédéric Millot, Béatrice Monlibert, Fabrice Monpoux, Despina Moshous, Luc Mouthon, Martine Munzer, Bénédicte Neven, Raphaëlle Nove-Josserand, Eric Oksenhendler, Marie Ouachée-Chardin, Caroline Oudot, Anne Pagnier, Jean-Louis Pasquali, Marlène Pasquet, Yves Perel, Capucine Picard, Christophe Piguet, Dominique Plantaz, Johan Provot, Pierre Quartier, Frédéric Rieux-Laucat, Pascal Roblot, Pierre-Marie Roger, Pierre-Simon Rohrlich, Hervé Rubie, Valéry Salle, Françoise Sarrot-Reynauld, Amélie Servettaz, Jean-Louis Stephan, Nicolas Schleinitz, Felipe Suarez, Laure Swiader, Sophie Taque, Caroline Thomas, Olivier Tournilhac, Caroline Thumerelle, François Tron, Jean-Pierre Vannier, Jean-François Viallard.
Funding
The CEREDIH is funded by the French Ministry of Health. It has received additional, unrestricted educational grants from companies (LFB, GSK, Pfizer, CSL Behring, Shire, Octapharma, and Binding Site) and patient associations (AT-Europe and Trophée Guillaume).
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Patients newly included in the CEREDIH/ESID database gave their consent to participation; patients who were already registered in the database had given their consent previously. The study’s objectives and procedures were approved by the local investigation review board (CPP Ile de France II, Paris, France) on June 7, 2010.
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Coignard-Biehler, H., Mahlaoui, N., Pilmis, B. et al. A 1-Year Prospective French Nationwide Study of Emergency Hospital Admissions in Children and Adults with Primary Immunodeficiency. J Clin Immunol 39, 702–712 (2019). https://doi.org/10.1007/s10875-019-00658-9
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DOI: https://doi.org/10.1007/s10875-019-00658-9