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Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies

2017; Elsevier BV; Volume: 140; Issue: 5 Linguagem: Inglês

10.1016/j.jaci.2016.12.978

1097-6825

Alain Fischer, Johan Provot, Jean-Philippe Jaı̈s, Alexandre Alcaïs, Nizar Mahlaoui, D. Adoué, Nathalie Aladjidi, Zahir Amoura, P. Arlet, Corinne Armari‐Alla, Brigitte Bader‐Meunier, Vincent Barlogis, Sophie Bayart, Beatrice Beaurain, Yves Bertrand, Boris Bienvenu, Stéphane Blanche, Damien Bodet, Bernard Bonnotte, Raphaël Borie, Patrick Boutard, Claire Briandet, Jean‐Paul Brion, Carolina Brito, Jacques Brouard, Émilie Catherinot, Olivia Chandesris, Sarah Beaussant-Cohen, Hélène Coignard-Biehler, Laurence Costes, Louis‐Jean Couderc, Gérard Couillault, Virginie Courteille, Elodie Curlier, Geneviève de Saint Basile, François Deméocq, Nathalie de Vergnes, Catherine Devoldère, Anne Deville, Jean Donadieu, Éric Doré, Fabienne Dulieu, I. Durieu, C. Edan, Natacha Entz Werle, Claire Fieschi, Fanny Fouyssac, Pierre Frange, Vincent Gajdos, Lionel Galicier, Virginie Gandemer, Martine Gardembas, C. Gaud, B. Grosbois, Gaëlle Guillerm, É. Hachulla, Mohamed Hamidou, Sébastien Heritier, Olivier Hermine, C. Hoarau, Bruno Hoen, A. Hot, S. Humbert, Arnaud Jaccard, Serge Jacquot, Jean-Philippe Jaı̈s, R. Jaussaud, Pierre‐Yves Jeandel, Éric Jeziorski, Kamila Kébaïli, Anne‐Sophie Korganow, Philippe Labrune, Olivier Lambotte, Fanny Lanternier, C. Larroche, Alain Le Quellec, Emmanuelle Le Moigne, Vincent Le Moing, Yvon Lebranchu, Marc Lecuit, Guillaume Lefèvre, Richard Lemal, Philippe Le Moine, Valérie Li Thiao Te, Olivier Lortholary, Patrick Lutz, Aude Magérus‐Chatinet, Marion Malphettes, Aude Marie‐Cardine, Nicolas Martin Silva, A. Masseau, Christian Massot, Françoise Mazingue, Étienne Merlin, Gérard Michel, Frédéric Millot, Odile Minckes, Béatrice Monlibert, Fabrice Monpoux, Despina Moshous, Luc Mouthon, Martine Münzer, Bénédicte Neven, Raphaëlle Nové-Josserand, Éric Oksenhendler, O Marie, Anne Pagnier, Jean‐Louis Pasquali, Marlène Pasquet, Isabelle Pellier, Yves Pérel, Antoinette Perlat, Capucine Pïcard, Christophe Piguet, Dominique Plantaz, Pierre Quartier, Frédéric Rieux‐Laucat, P. Roblot, Pierre‐Marie Roger, Pierre‐Simon Rohrlich, Bruno Royer, V. Salle, F. Sarrot-Reynauld, Amélie Servettaz, Jean-Louis Stéphan, N. Schleinitz, Felipe Suárez, L. Swiader, Sophie Taque, Caroline Thomas, Olivier Tournilhac, C. Thumerelle, Jean‐Pierre Vannier, Jean‐François Viallard,

Blood disorders and treatments

Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerable increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases, including allergy, autoimmunity, and inflammation.We aimed at determining the incidence of autoimmunity and inflammation in patients with PIDs.We have retrospectively screened 2183 consecutive cases of PID in the Centre de Référence Déficits Immunitaires Héréditaires registry (CEREDIH; the French national PID registry) for the occurrence of autoimmunity and inflammation.One or more autoimmune and inflammatory complications were noted in 26.2% of patients, with a risk of onset throughout the patient's lifetime. The risk of autoimmune cytopenia was at least 120 times higher than in the general population, the risk of inflammatory bowel disease in children was 80 times higher, and the risk of other autoimmune manifestations was approximately 10 times higher. Remarkably, all types of PIDs were associated with a risk of autoimmune and inflammatory complications, although the greatest risk was associated with T-cell PIDs and common variable immunodeficiency. The occurrence of autoimmune disease is a negative prognostic factor for survival.Our results provide the basis for a detailed prospective evaluation of autoimmunity and inflammation in the context of PIDs, with a view to accurately assessing these risks and describing the possible effect of medical intervention.