Current Spectrum of Infections in Patients with X-Linked Agammaglobulinemia.

Agammaglobulinemia / immunology Child, Preschool Cross-Sectional Studies Female Genetic Diseases, X-Linked / immunology Humans Immunoglobulin G / immunology Immunoglobulins, Intravenous / immunology Immunosuppressive Agents / immunology Infant Infections / immunology Male Retrospective Studies
Bruton’s disease X-linked agammaglobulinemia agammaglobulinemia immunoglobulin replacement therapy

Journal

Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137

Informations de publication

Date de publication:
08 2021
Historique:
received: 21 02 2021
accepted: 12 04 2021
pubmed: 22 4 2021
medline: 4 2 2022
entrez: 21 4 2021
Statut: ppublish

Résumé

Outcome of patients with X-linked agammaglobulinemia (XLA) has improved with the widespread use of immunoglobulin replacement therapy (IgRT). There are few data on the spectrum of infections experienced by patients undergoing IgRT. We carried out a retrospective cross-sectional analysis of the records of XLA patients seen at Necker-Enfants Malades Hospital, Paris. For each infection, we evaluated infection site, microbial etiology, antibiotic prophylaxis, immunosuppressive treatment, IgRT route, and last known IgG trough level. Sixty patients were included, who cumulated a follow-up of 1470 patient-years. We recorded 188 infections, including 97 after initiation of IgRT. The rate of infection was highest before IgRT (0.66 vs. 0.06 per person-year (ppy), p < 0.001) and was higher after the age of 16 compared to before (0.14 vs. 0.05 ppy, p = 0.048). It was similar for patients receiving intravenous or subcutaneous Ig (0.09 vs 0.05 ppy, p = 0.54). The lungs and gastrointestinal tract accounted for 71% of infection sites. Forty-six (47%) infections occurred in patients receiving antibiotic prophylaxis. Sixteen (16.5%) infections occurred in patients receiving immunosuppressive therapy, which more frequently occurred after age 16 (35% vs. 2.4%, p < 0.001). The median IgG trough level prior to all infections was 8.4 g/L. Almost half (44.3%) of infections occurred with prior IgG trough levels > 8 g/L, and 16/97 (16.7%) in patients with trough levels > 10 g/L. Infection remains a significant issue in patients with XLA undergoing IgRT despite adequate IgG trough levels. Chronic inflammatory manifestations of X-linked agammaglobulinemia and immunosuppressive therapies may be significant drivers of infection during adulthood.

Identifiants

DOI: 10.1007/s10875-021-01043-1 PMID: 33880703
pubmed: 33880703
doi: 10.1007/s10875-021-01043-1
pii: 10.1007/s10875-021-01043-1
doi:

Substances chimiques

Immunoglobulin G 0
Immunoglobulins, Intravenous 0
Immunosuppressive Agents 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1266-1271

Informations de copyright

© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.

Références

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Auteurs

Olivier Paccoud (O)

Necker-Pasteur Center for Infectious Diseases and Tropical Medicine, Necker-Enfants Malades University Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Université de Paris, 149 Rue de Sèvres, 75015, Paris, France. olivier.paccoud@aphp.fr.
ORCID: 0000-0002-3975-3136

Nizar Mahlaoui (N)

Pediatric Immuno-Hematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, AP-HP, Université de Paris, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

Despina Moshous (D)

Pediatric Immuno-Hematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, AP-HP, Université de Paris, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

Claire Aguilar (C)

Necker-Pasteur Center for Infectious Diseases and Tropical Medicine, Necker-Enfants Malades University Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Université de Paris, 149 Rue de Sèvres, 75015, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.

Bénédicte Neven (B)

Pediatric Immuno-Hematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, AP-HP, Université de Paris, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

Fanny Lanternier (F)

Necker-Pasteur Center for Infectious Diseases and Tropical Medicine, Necker-Enfants Malades University Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Université de Paris, 149 Rue de Sèvres, 75015, Paris, France.

Felipe Suarez (F)

Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.
Department of Hematology, Necker-Enfants Malades University Hospital, AP-HP, Université de Paris, Paris, France.

Capucine Picard (C)

Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.
Study Center for Primary Immunodeficiencies (CEDI), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

Alain Fischer (A)

Pediatric Immuno-Hematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, AP-HP, Université de Paris, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

Stéphane Blanche (S)

Pediatric Immuno-Hematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, AP-HP, Université de Paris, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.

Marc Lecuit (M)

Necker-Pasteur Center for Infectious Diseases and Tropical Medicine, Necker-Enfants Malades University Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Université de Paris, 149 Rue de Sèvres, 75015, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
Biology of Infection Unit, Inserm U1117, Pasteur Institute, Paris, France.

Olivier Hermine (O)

Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.
French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker-Enfants Malades University Hospital, AP-HP, Paris, France.
Department of Hematology, Necker-Enfants Malades University Hospital, AP-HP, Université de Paris, Paris, France.

Olivier Lortholary (O)

Necker-Pasteur Center for Infectious Diseases and Tropical Medicine, Necker-Enfants Malades University Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Université de Paris, 149 Rue de Sèvres, 75015, Paris, France.
Imagine Institute, INSERM UMR1163, Université de Paris, Paris, France.

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Classifications MeSH

questionsmedicales.fr › Maladies du système immunitaire › Déficits immunitaires › Agammaglobulinémie › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Personnes › Tranches d'âge › Enfant › Enfant d'âge préscolaire › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études transversales › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Maladies génétiques congénitales › Maladies génétiques liées au chromosome X › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Globulines › Sérum-globulines › Immunoglobulines › Anticorps › Isotypes des immunoglobulines › Immunoglobuline G › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Acides aminés, peptides et protéines › Protéines › Globulines › Sérum-globulines › Immunoglobulines › Anticorps › Immunoglobulines par voie veineuse › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Effets physiologiques des médicaments › Facteurs immunologiques › Immunosuppresseurs › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Personnes › Tranches d'âge › Nourrisson › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Infection › Current Spectrum of Infections in Patients...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études de cohortes › Études rétrospectives › Current Spectrum of Infections in Patients...