From the Guidelines
Common Variable Immunodeficiency (CVID) in the gastrointestinal (GI) tract is a primary immunodeficiency disorder that affects approximately 20-25% of patients, causing various GI complications such as chronic gastritis, lymphoid nodular hyperplasia, villous atrophy, inflammatory bowel disease, and enteropathy, as noted in the practice parameter for the diagnosis and management of primary immunodeficiency 1.
GI Complications in CVID
The GI complications in CVID patients are diverse and can include:
- Chronic gastritis with or without pernicious anemia
- Lymphoid nodular hyperplasia
- Villous atrophy
- Inflammatory bowel disease
- Enteropathy
- Giardiasis and enteritis with C jejuni and salmonellosis
- Chronic viral enteritis caused by CMV, norovirus, or parechovirus
Diagnosis and Management
Diagnosis of GI complications in CVID typically involves endoscopy with biopsies showing characteristic findings such as villous atrophy, increased intraepithelial lymphocytes, or nodular lymphoid hyperplasia. Treatment focuses on immunoglobulin replacement therapy (typically IVIG or SCIG) to restore antibody levels, targeted antimicrobial therapy for specific infections, and management of inflammatory complications with corticosteroids or other immunosuppressants when necessary, as recommended in the practice parameter for the diagnosis and management of primary immunodeficiency 1.
Monitoring and Follow-up
Regular monitoring of gastrointestinal status is essential in patients with CVID, as stated in Summary statement 89 of the practice parameter for the diagnosis and management of primary immunodeficiency 1. This includes monitoring for possible autoimmune diseases, nonmalignant and malignant lymphoproliferative disease, and other complications associated with CVID.
From the Research
Definition and Characteristics of CVID
- Common Variable Immunodeficiency (CVID) is a primary antibody deficiency characterized by impaired production of specific immunoglobulins, particularly IgG and IgA 2, 3, 4.
- It is the most common symptomatic primary immunodeficiency in adults, with heterogeneous and unspecific symptoms 3.
- CVID patients have an increased susceptibility to infections, autoimmunity, polyclonal lymphoproliferation, and malignancies 2, 4.
Clinical Manifestations of CVID
- Recurrent bacterial infections, lymphoproliferation, autoimmunity, pulmonary disease, liver disease, enteropathy, granulomas, and an increased risk of malignancy are common clinical manifestations of CVID 4.
- Gastrointestinal (GI) involvement, including enteropathy, is a significant aspect of CVID, highlighting the importance of considering CVID in the differential diagnosis of GI disorders 4.
Treatment and Management of CVID
- Lifelong immunoglobulin replacement therapy is the primary treatment for CVID, which helps prevent the majority of infections when given regularly 5, 4.
- Other treatments, such as antibiotics, immunosuppressants, and hematopoietic stem cell transplantation, may be used to manage specific complications of CVID 3.
- Rituximab, an anti-CD20 monoclonal antibody, has been used to treat autoimmune and non-malignant lymphoproliferative manifestations associated with CVID, with promising results 2.