Title :

Diagnosis, Characteristics, and Outcome of Selective Anti-polysaccharide Antibody Deficiencies In A Retrospective Cohort of 55 Adult Patients.

Author(s) :

Perrard, Nicolas [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Stabler, Sarah [Auteur]
Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 [CIIL]
Sanges, Sebastien [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Terriou, Louis [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Lamblin, Catherine [Auteur]
Ramsay Générale de Santé - Hôpital Privé La Louvière
Gaillard, Sacha [Auteur]
Vuotto, Fanny [Auteur]
Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 [CIIL]
Chenivesse, Cecile [Auteur]
Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204 [CIIL]
Mortuaire, Geoffrey [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Batteux, Frédéric [Auteur]
Servie d'immunologie biologique [CHU Cochin]
Mirgot, Floriane [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Collet, Aurore [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Lopez, Benjamin [Auteur]
Dubucquoi, Sylvain [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Labalette, Myriam [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Hachulla, Eric [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Launay, David [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Lefevre, Guillaume [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]

Journal title :

Journal of Clinical Immunology

Abbreviated title :

J Clin Immunol

Volume number :

45

Pages :

82

Publisher :

Springer Nature Link

Publication date :

2025-03-22

ISSN :

1573-2592

English keyword(s) :

Specific antibody deficiency
Selective anti-polysaccharide antibody deficiency
Primary immunodeficiency
Encapsulated bacterial infections
Preventive antibiotherapy
Immunoglobulin replacement therapy
Asthma

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Selective anti-polysaccharide antibody deficiency (SPAD) predisposes to encapsulated bacterial infections. The diagnosis is challenging, and literature reports are scarce in adult patients, we therefore aim to describe the ...
Show more >Selective anti-polysaccharide antibody deficiency (SPAD) predisposes to encapsulated bacterial infections. The diagnosis is challenging, and literature reports are scarce in adult patients, we therefore aim to describe the demographics, infectious complications, therapeutic strategies, and outcome of adult patients. We conducted a multicenter observational study involving 55 adult patients with SPAD. The median [interquartile range, IQR] age was 45 [36–60] years at diagnosis of SPAD, and 75% of patients were female. Twenty-one patients (38%) had a history of allergic and/or inflammatory disease, mainly asthma (n = 12), and rheumatic diseases (n = 6). Twelve patients (22%) were diagnosed after a single severe infection and 43 (78%) in a context of recurrent benign and/or severe infections. In the latter, the median time from first infections to diagnosis was 74.5 [33–167] months. Diagnostic delay was significantly higher in patients presenting with bronchiectasis than in those without (122 months [33–219.5] vs 24 months [14.5–74.5], p = 0.0042). In 22 patients (40%) receiving immunoglobulin replacement therapy (IgRT), the mean (min–max) frequency of antibiotic courses decreased from 7.9 (2–18) to 0.7 (0–2) courses per year (p < 0.001) with a median follow-up period of 46 [27–73] months. Patients diagnosed after a single severe infection did not have any relapse during a median follow-up of 85 [80.5–104.5] months after diagnosis. Adult patients with SPAD have allergic or inflammatory disorders which could contribute to the diagnostic delay. IgRT is effective in preventing recurrent infections. Further studies are warranted to confirm if SPAD should be considered after a first unexplained severe bacterial infection.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2025-03-28T22:00:33Z
2025-04-09T06:12:33Z