A High Prevalence of Gastrointestinal Manifestations in Common Variable Immunodeficiency

• Published in: The American journal of gastroenterology Vol. 114; no. 4; pp. 648 - 655
• Main Authors: Pikkarainen, Sampsa, Martelius, Timi, Ristimäki, Ari, Siitonen, Sanna, Seppänen, Mikko R J, Färkkilä, Martti
• Format: Journal Article
• Language: English
• Published: United States Wolters Kluwer 01.04.2019
• Subjects: Biomarkers - metabolism; Common Variable Immunodeficiency - complications; Female; Finland - epidemiology; Gastrointestinal Diseases - epidemiology; Gastrointestinal Diseases - etiology; Gastrointestinal Diseases - immunology; Humans; Immunophenotyping; Male; Middle Aged; Prevalence; Finland
• ISSN: 0002-9270; 1572-0241
• DOI: 10.14309/ajg.0000000000000140

Common variable immunodeficiency (CVID) is associated with a spectrum of autoimmune complications. We studied the prevalence of gastrointestinal (GI) manifestations and infections in patients with CVID. Complete clinical data of 132 Finnish patients with CVID (106 probable and 26 possible CVID) followed up between 2007 and 2016 were collected to a structured database. Data on endoscopies, histology, and laboratory studies were retrieved from patient files. Most common referral indications were diarrhea and/or weight loss (47%-67%). Patients with probable CVID had higher fecal calprotectin and α1-antitrypsin and lower blood vitamin B12 than patients with possible CVID. Gastroscopy and colonoscopy were done to 71 (67%) and 63 (59%) patients with probable CVID, respectively. Endoscopies showed that 15% of them had chronic active gastritis and 17% atrophic gastritis and 3% had gastric adenocarcinoma. A celiac sprue-like condition was found in 7 patients (10%), of whom 3 responded to a gluten-free diet. Colonoscopies demonstrated unspecific colitis (14%), ulcerative colitis (8%), microscopic colitis (10%), and Crohn's disease (2%). Colonic polyps were noted in 30% of patients, and 3% had lower GI malignancies. Thirty-five patients with CVID had bacterial or parasitic gastroenteritis; chronic norovirus was detected in 4 patients with probable CVID. Patients with GI inflammation had higher levels of fecal calprotectin and blood CD8 T lymphocytes but lower counts of CD19CD27 memory B cells and/or CD19 B cells. Immunophenotype with low B-cell counts was associated with higher fecal calprotectin levels. Patients with CVID had a high prevalence of GI manifestations and infections of the GI tract. GI inflammation was associated with a distinct immunophenotype and elevated fecal calprotectin.