From the Guidelines
Yes, Common Variable Immunodeficiency (CVID) can be associated with lymphocytic panenteritis later in life.
Testing and Diagnosis
- Gastrointestinal status should be monitored regularly in patients with CVID, as approximately 20% to 25% of patients have gastrointestinal complications, including chronic gastritis, lymphoid nodular hyperplasia, villous atrophy, inflammatory bowel disease, and enteropathy 1.
- Testing for lymphocytic panenteritis in CVID patients may involve endoscopy, biopsy, and imaging studies to evaluate the extent of gastrointestinal involvement.
- Patients with CVID are also at risk of developing autoimmune diseases, including autoimmune cytopenias, and nonmalignant and malignant lymphoproliferative disease, which should be monitored and treated accordingly 1.
Management
- CVID should be managed aggressively with antimicrobials, IgG replacement, and careful attention to pulmonary status, as infectious lung disease occurs in the majority of patients 1.
- Patients with CVID may benefit from the addition of antibiotic prophylaxis to their maintenance regimen for periods of months or years or permanently, especially those with frequent bronchitis and pneumonia 1.
- Stem cell transplantation can be considered for patients with CVID with malignancy or severe organ damage, although experience is limited and outcomes are variable 1.
From the Research
Association between CVID and Lymphocytic Panenteritis
- CVID is a heterogeneous collection of disorders of immune dysregulation, and approximately 70% of patients have one or more non-infectious complications, including enteropathy 2.
- Gastrointestinal diseases are a major cause of morbidity in CVID, and diagnosis remains challenging due to similarities with other conditions such as celiac disease and inflammatory bowel disease (IBD) 3.
- Lymphocytic panenteritis is not explicitly mentioned in the provided studies, but lymphocytic sprue/colitis-like patterns were observed in some CVID patients, suggesting a possible association 3.
Testing for CVID and Lymphocytic Panenteritis
- Diagnosis of CVID is based on criteria established by the European Society for Immunodeficiencies and Pan-American Group for Immunodeficiency (ESID/PAGID), which includes clinical and laboratory evaluations 4.
- Upper gastrointestinal endoscopy and random biopsies from the gastric antrum and descending duodenum can help identify gastro-duodenal histopathological findings, including chronic active gastritis, chronic duodenitis, and intraepithelial lymphocytosis 5.
- Flow cytometry and CD4+ T-cell proliferation tests can also be used to evaluate lymphocyte subsets and functional abnormalities in CVID patients 6.
- Histological features of CVID in the gut can be diverse and may include IBD-like, celiac disease-like, and GVHD-like patterns, emphasizing the importance of adequate clinical information and awareness of atypical histological features 3.