Title :

Protein-losing Enteropathy as a Complication and/or Differential Diagnosis of Common Variable Immunodeficiency.

Author(s) :

Sanges, Sébastien [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France [CeRAINO]
Germain, Nicolas [Auteur]
Vignes, Stéphane [Auteur]
Hôpital Cognacq-Jay
Seguy, David [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Stabler, Sarah [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Etienne, Nicolas [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France [CeRAINO]
Terriou, Louis [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Launay, David [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Huglo, Damien [Auteur]
Thérapies Lasers Assistées par l'Image pour l'Oncologie (ONCO-THAI) - U1189
Dubucquoi, Sylvain [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Labalette, Myriam [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Lefevre, Guillaume [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Journal title :

Journal of Clinical Immunology

Abbreviated title :

J Clin Immunol

Publication date :

2022-06-24

ISSN :

1573-2592

English keyword(s) :

Protein-losing enteropathy
Common variable immunodeficiency
Lymphopenia
Hypogammaglobulinemia
Antibody deficiency
Primary intestinal lymphangiectasia

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

As protein-losing enteropathy (PLE) can lead to hypogammaglobulinemia and lymphopenia, and since common variable immunodeficiency (CVID) is associated with digestive complications, we wondered if (1) PLE could occur during ...
Show more >As protein-losing enteropathy (PLE) can lead to hypogammaglobulinemia and lymphopenia, and since common variable immunodeficiency (CVID) is associated with digestive complications, we wondered if (1) PLE could occur during CVID and (2) specific features could help determine whether a patient with antibody deficiency has CVID, PLE, or both. Eligible patients were thus classified in 3 groups: CVID + PLE (n = 8), CVID-only (= 19), and PLE-only (n = 13). PLE was diagnosed using fecal clearance of α1-antitrypsin or 111In-labeled albumin. Immunoglobulin (Ig) A, G, and M, naive/memory B and T cell subsets were compared between each group. CVID + PLE patients had multiple causes of PLE: duodenal villous atrophy (5/8), nodular follicular hyperplasia (4/8), inflammatory bowel disease-like (4/8), portal hypertension (4/8), giardiasis (3/8), and pernicious anemia (1/8). Compared to the CVID-only group, CVID + PLE patients had similar serum Ig levels, B cell subset counts, but lower naive T cell proportion and IgG replacement efficiency index. Compared to the CVID-only group, PLE-only patients did not develop infections but had higher serum levels of IgG (p = 0.03), IgA (p < 0.0001), and switched memory B cells (p = 0.001); and decreased naive T cells (CD4+: p = 0.005; CD8+: p < 0.0001). Compared to the PLE-only group, CVID + PLE patients had higher infection rates (p = 0.0003), and lower serum Ig (especially IgA: p < 0.001) and switched memory B cells levels. In conclusion, PLE can occur during CVID and requires higher IgG replacement therapy dosage. PLE can also mimic CVID and is associated with milder immunological abnormalities, notably mildly decreased to normal serum IgA and switched memory B cell levels.Show less >

Language :

Anglais

Peer reviewed article :

Oui

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 :

2024-01-12T01:12:28Z
2024-03-08T10:15:03Z