Learn about Common Variable Immunodeficiency (CVID), also known as hypogammaglobulinemia. This resource provides information on CVID diagnosis, symptoms, treatment, and management. Find details relevant to healthcare professionals, including clinical documentation, medical coding, and ICD codes for CVID. Explore resources for patients and families seeking information on living with CVID and its associated health challenges.
Also known as
Common variable immunodeficiency
Defect in antibody production causing increased susceptibility to infections.
Disorders of the immune mechanism
Conditions affecting the body's ability to fight disease.
Antibody deficiencies
Low levels of antibodies leading to impaired immune response.
Follow this step-by-step guide to choose the correct ICD-10 code.
Is the diagnosis Common Variable Immunodeficiency (CVID)?
Yes
Is there antibody deficiency with near-normal B cell count?
No
Do NOT code D83.4. Review documentation for alternative diagnosis.
When to use each related code
| Description |
|---|
| Antibody deficiency with recurrent infections. |
| Selective IgA deficiency, often asymptomatic. |
| Low IgG with varying severity and manifestations. |
Coding CVID without specifying the type (e.g., late-onset, atypical) can lead to inaccurate reimbursement and data analysis.
Insufficient documentation of specific antibody deficiencies may cause downcoding or claim denials. CDI review crucial.
Misdiagnosis or miscoding CVID as other similar conditions (e.g., XLA) impacts quality reporting and reimbursements.
Q: What are the most effective diagnostic criteria for Common Variable Immunodeficiency (CVID) in adult patients, and how can I differentiate it from other hypogammaglobulinemia conditions?
A: Diagnosing CVID in adults requires a multifaceted approach. The key criteria include decreased serum IgG levels at least two standard deviations below the age-adjusted mean, decreased IgA and/or IgM, poor or absent antibody response to vaccines (e.g., pneumococcal, tetanus), and exclusion of other causes of hypogammaglobulinemia like X-linked agammaglobulinemia (XLA) or secondary immunodeficiencies. Differentiation from other conditions relies heavily on family history, genetic testing (for XLA or specific gene defects), and assessment of B cell numbers and function. While reduced or absent isotype-switched memory B cells can be indicative of CVID, it's not always definitive. Consider implementing a comprehensive immunological workup including lymphocyte subset analysis and specific antibody titers post-vaccination. Explore how genetic testing can aid in confirming CVID and ruling out other primary immunodeficiencies. Further investigation into the patient's clinical presentation, including recurrent infections, autoimmune manifestations, and granulomatous disease, is crucial for accurate diagnosis. Learn more about the diagnostic challenges and pitfalls in CVID.
Q: What are the current best practices for managing infections and complications in patients with CVID, specifically focusing on immunoglobulin replacement therapy and prophylactic antibiotics?
A: Management of CVID hinges on minimizing infection risk and treating complications. Intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG) replacement therapy remains the cornerstone of treatment, aiming to achieve adequate IgG trough levels. The optimal dosing and frequency should be individualized based on patient response, infection frequency, and trough levels. Prophylactic antibiotics may be considered in select patients with recurrent bacterial infections despite immunoglobulin replacement, particularly in those with structural lung disease like bronchiectasis. However, long-term antibiotic use carries risks, including the development of antibiotic resistance. Therefore, antibiotic prophylaxis should be used judiciously and tailored to the individual patient's needs. Consider implementing a patient-specific monitoring plan to assess the effectiveness of immunoglobulin therapy and identify early signs of infection. Explore how pulmonary function tests, imaging studies, and regular clinical assessments can guide management decisions. Learn more about the role of other supportive therapies, including nutritional support and management of associated autoimmune and granulomatous complications.
Patient presents with a history of recurrent bacterial infections, primarily sinopulmonary infections such as pneumonia, bronchitis, and sinusitis. The patient also reports chronic diarrhea and fatigue. Given the recurrent infections and clinical presentation, common variable immunodeficiency (CVID) is suspected. Laboratory evaluation reveals significantly reduced serum immunoglobulin levels, including IgG, IgA, and often IgM, meeting the diagnostic criteria for CVID. Other potential causes of hypogammaglobulinemia, such as secondary immunodeficiency, have been ruled out through comprehensive medical history, physical examination, and appropriate laboratory testing. Differential diagnoses considered include X-linked agammaglobulinemia, IgA deficiency, and other primary immunodeficiencies. The patient's family history is non-contributory for primary immunodeficiencies. Current treatment plan includes immunoglobulin replacement therapy with intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (SCIG) to prevent further infections and improve quality of life. Patient education regarding infection prevention strategies, including hand hygiene and vaccination recommendations for individuals with CVID, has been provided. Regular monitoring of immunoglobulin levels and response to therapy will be conducted. Ongoing evaluation for potential complications of CVID, such as autoimmune diseases, granulomatous disease, and lymphoid malignancy, is warranted. The patient demonstrates understanding of the diagnosis, management plan, and importance of follow-up care.