What are the symptoms of Common Variable Immunodeficiency (CVID)?

From the Guidelines

Patients with Common Variable Immunodeficiency (CVID) typically present with recurrent infections, particularly of the respiratory tract, including sinusitis, bronchitis, and pneumonia, as well as gastrointestinal issues and autoimmune disorders. The symptoms of CVID can be diverse and affect various organ systems. According to the practice parameter for the diagnosis and management of primary immunodeficiency 1, infectious lung disease occurs in the majority of patients, with a clinical presentation similar to allergic asthma, and noninfectious chronic pulmonary disease is associated with reduced survival.

Key Symptoms

• Recurrent sinusitis, otitis media, and bronchitis
• Bronchiectasis, a common pulmonary complication, occurring in 10% to 20% of patients
• Granulomatous and lymphocytic interstitial lung disease (GLILD), found in approximately 10% of patients, which can be associated with increased mortality
• Gastrointestinal complications, including chronic gastritis, lymphoid nodular hyperplasia, villous atrophy, inflammatory bowel disease, and enteropathy, affecting approximately 20% to 25% of patients
• Autoimmune diseases, with an overall prevalence of approximately 20%, including autoimmune cytopenias, rheumatoid arthritis, and thyroid disease
• Lymphadenopathy and splenomegaly, frequently observed in patients with CVID
• Increased risk of certain cancers, especially lymphomas and gastric cancers

Disease Management

The management of CVID should be aggressive, with antimicrobials, IgG replacement, and careful attention to pulmonary status, as stated in the practice parameter 1. Patients often benefit from the addition of antibiotic prophylaxis to their maintenance regimen for periods of months or years or permanently. Regular monitoring of gastrointestinal status and vigilance for possible autoimmune diseases and nonmalignant and malignant lymphoproliferative disease are also crucial in the follow-up of patients with CVID.

From the Research

Symptoms of Common Variable Immunodeficiency (CVID)

The symptoms of CVID can be diverse and include:

• Recurrent sino-pulmonary infections 2
• Recurrent bacterial infections, such as pneumonia and sinus infections 3, 4
• Autoimmune diseases, such as:
  • Idiopathic thrombocytopenia purpura (ITP) 2, 5, 6
  • Autoimmune hemolytic anemia 5, 6
  • Sarcoid-like granulomatous disease 5
  • Gastrointestinal inflammation 5
• Intestinal pseudo-obstruction, which has been reported in children with CVID, but is rare in adults 2
• Peripheral mononeuropathy, which is a rare finding in CVID 3
• Malignancy 4
• Severe pseudomonas pneumonia 2
• Nausea, vomiting, and abdominal distension 2
• Left leg and lower back pain, left foot drop, and decreased sensation over the left leg 3

Clinical Manifestations

CVID can present with a broad range of clinical manifestations, making diagnosis challenging 2, 3, 4. The disease can be characterized by hypogammaglobulinemia, impaired antibody response to vaccines, and recurrent infections 2, 3.

Autoimmune and Inflammatory Complications

Autoimmune and inflammatory complications are common in CVID, and can include autoimmune cytopenias, such as immune thrombocytopenia, hemolytic anemia, or neutropenia 5, 6. These complications can be the initial manifestation of CVID, and may recur over time 6.