Prevention of Infections During Primary Immunodeficiency

Claire Aguilar (1, 2) , Marion Malphettes (3, 2) , Jean Donadieu (4, 2) , Olivia Chandesris (2, 5) , Hélène Coignard-Biehler (2, 1, 5) , Emilie Catherinot (2, 6) , Isabelle Pellier (2, 7) , Jean-Louis Stéphan (2, 8) , Vincent Le Moing (9, 2) , Vincent Barlogis (10, 2) , Felipe Suarez (2, 5, 11) , Stéphane Gérart (5) , Fanny Lanternier (2, 5, 1) , Arnaud Jaccard (12, 2) , Paul-Henri Consigny (1) , Florence Moulin (11) , Odile Launay (13) , Marc Lecuit (11, 14, 15, 16, 2) , Olivier Hermine (15, 16, 2) , Eric Oksenhendler (2, 17) , Capucine Picard (2, 11, 16) , Stéphane Blanche (18, 2, 16) , Alain Fischer (2, 16, 11, 19) , Nizar Mahlaoui (2, 16, 11) , Olivier Lortholary (1, 2, 16)

1 Centre d'infectiologie Necker-Pasteur [CHU Necker]
2 CEREDIH - Centre de Référence Déficits Immunitaires Héréditaires
3 Service d'Immunopathologie [Hôpital Saint-Louis, Paris]
4 Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau]
5 IMAGINE - U1163 - Imagine - Institut des maladies génétiques
6 Hôpital Foch [Suresnes]
7 CHU Angers - Centre Hospitalier Universitaire d'Angers
8 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
9 Département Maladies Infectieuses et Tropicales, Hôpital Universitaire, Montpellier, France
10 Pédiatrie et oncologie pédiatrique [Hôpital de la Timone - APHM]
11 CHU Necker - Enfants Malades [AP-HP]
12 CHU Dupuytren
13 CIC 1417 - CIC Cochin Pasteur
14 Service des Maladies infectieuses et tropicales [CHU Necker]
15 UPD5 - Université Paris Descartes - Paris 5
16 Imagine - U1163 - Imagine - Institut des maladies génétiques (IHU)
17 Hôpital Saint-Louis
18 Service d'immuno-hématologie pédiatrique [CHU Necker]
19 Collège de France - Chaire Médecine expérimentale (A. Fischer)

Because infectious diseases are a major source of morbidity and mortality in the majority of patients with primary immunodeficiencies (PIDs), the application of a prophylactic regimen is often necessary. However, because of the variety of PIDs and pathogens involved, and because evidence is scarce, practices are heterogeneous. To homog-enize practices among centers, the French National Reference Center for PIDs aimed at elaborating recommendations for anti-infectious prophylaxis for the most common PIDs. We performed a literature review of infectious complications and prophylactic regimens associated with the most frequent PIDs. Then, a working group including different specialists systematically debated about chemoprophylaxis, immunotherapy, immunization, and recommendations for patients. Grading of prophylaxis was done using strength of recommendations (decreasing from A to D) and evidence level (decreasing from I to III). These might help infectious diseases specialists in the management of PIDs and improving the outcome of patients with PIDs.

Keywords: prophylaxis; primary immunodeficiency; immunoglobulins; immunizations

Domains: Life Sciences [q-bio]; Human health and pathology; Infectious diseases; Immunology

Claire Aguilar, Marion Malphettes, Jean Donadieu, Olivia Chandesris, Hélène Coignard-Biehler, et al.. Prevention of Infections During Primary Immunodeficiency. Clinical Infectious Diseases, 2014, 59 (10), pp.1462-1470. ⟨10.1093/cid/ciu646⟩. ⟨pasteur-02454019⟩