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

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 an...

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Bibliographic Details
Published in:Journal of clinical immunology 2022-10, Vol.42 (7), p.1461-1472
Main Authors: Sanges, Sébastien, Germain, Nicolas, Vignes, Stéphane, Séguy, David, Stabler, Sarah, Etienne, Nicolas, Terriou, Louis, Launay, David, Hachulla, Éric, Huglo, Damien, Dubucquoi, Sylvain, Labalette, Myriam, Lefèvre, Guillaume
Format: Article
Language:English
Subjects:
a1-antitrypsin
Atrophy
Biomedical and Life Sciences
Biomedicine
CD4 antigen
CD8 antigen
Common variable immunodeficiency
Common Variable Immunodeficiency - complications
Common Variable Immunodeficiency - diagnosis
Deficiency diseases
Diagnosis, Differential
Differential diagnosis
Giardiasis
Humans
Hyperplasia
Hypogammaglobulinemia
Immune system
Immunoglobulin A
Immunoglobulin G
Immunological memory
Immunology
Infectious Diseases
Inflammatory bowel diseases
Internal Medicine
Life Sciences
Lymphocytes B
Lymphocytes T
Lymphopenia
Medical Microbiology
Memory cells
Original Article
Pernicious anemia
Protein-Losing Enteropathies - complications
Protein-Losing Enteropathies - etiology
Serum levels
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Summary: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  
ISSN:0271-9142
1573-2592
DOI:10.1007/s10875-022-01299-1