Journal Article

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

Hachulla, Éric, Dubucquoi, Sylvain, Vignes, Stéphane, Launay, David, Labalette, Myriam, Lefèvre, Guillaume, Séguy, David, Stabler, Sarah, Etienne, Nicolas, Germain, Nicolas, Huglo, Damien, Terriou, Louis, Sanges, Sébastien

2022

Overview

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.

• Publisher: Springer US,Springer Nature B.V,Springer Verlag
• Subject: a1-antitrypsin / albumins / anemia / antibodies / Atrophy / B-lymphocytes / Biomedical and Life Sciences / Biomedicine / blood serum / CD4 antigen / CD8 antigen / Common variable immunodeficiency / Common Variable Immunodeficiency - complications / Common Variable Immunodeficiency - diagnosis / Deficiency diseases / Diagnosis, Differential / Differential diagnosis / digestive system diseases / Giardiasis / Humans / Hyperplasia / Hypogammaglobulinemia / Immune system / Immunoglobulin A / Immunoglobulin G / immunoglobulins / Immunological memory / Immunology / immunosuppression / Infectious Diseases / Inflammatory bowel diseases / Internal Medicine / Life Sciences / Lymphocytes B / Lymphocytes T / Lymphopenia / Medical Microbiology / memory / Memory cells / Original Article / patients / Pernicious anemia / portal hypertension / Protein-Losing Enteropathies - complications / Protein-Losing Enteropathies - etiology / Serum levels / T-lymphocytes / therapeutics