D83

Common variable immunodeficiency

Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by significantly low levels of serum immunoglobulins (IgG, IgA, and often IgM) and a failure to produce specific antibodies in response to infections or vaccines. It is the most common clinically significant primary immunodeficiency, often diagnosed in the second to fourth decades of life, though it can occur at any age. The condition arises from defects in B-cell differentiation and maturation into plasma cells, though T-cell abnormalities are also frequently present. Clinically, it manifests as a heterogeneous syndrome; while many patients suffer primarily from recurrent sinopulmonary bacterial infections, a significant subset develops non-infectious complications including autoimmune disorders, granulomatous disease, lymphoid hyperplasia, and an increased risk of malignancies, particularly lymphomas and gastric cancer.

Clinical Symptoms

  • Recurrent pneumonia
  • Chronic or recurrent sinusitis
  • Recurrent otitis media
  • Bronchiectasis (secondary to chronic lung infections)
  • Chronic diarrhea
  • Malabsorption and weight loss
  • Giardiasis infections
  • Splenomegaly
  • Lymphadenopathy
  • Autoimmune hemolytic anemia
  • Immune thrombocytopenic purpura (ITP)
  • Granulomatous lymphocytic interstitial lung disease (GLILD)
  • Inflammatory bowel disease (IBD)-like symptoms
  • Nodular lymphoid hyperplasia
  • Frequent skin infections or abscesses
  • Failure to thrive in pediatric presentations

Common Causes

  • Failure of B-cell differentiation into immunoglobulin-secreting plasma cells
  • Genetic mutations in TNFRSF13B (TACI) gene
  • Genetic mutations in ICOS (Inducible T-cell Co-stimulator) gene
  • Genetic mutations in TNFRSF13C (BAFF-R) gene
  • CD19, CD20, CD21, or CD81 complex deficiencies
  • NFKB2 gene mutations
  • CTLA4 haploinsufficiency
  • LRBA deficiency
  • Polygenic inheritance (in approximately 80-90% of cases the exact genetic cause remains unidentified)
  • T-cell signaling defects leading to impaired B-cell help

Documentation & Coding Tips

Document specific B-cell and T-cell phenotypes to support code selection at the fourth-character level.

Example: Patient with common variable immunodeficiency (D83.0) presenting with recurrent pneumonia and significantly reduced CD19+ B-cell counts (less than 1 percent of total lymphocytes). This chronic condition requires ongoing intravenous immunoglobulin replacement therapy to prevent life-threatening infections. Risk adjustment reflects the increased susceptibility to opportunistic pathogens and the need for complex multidisciplinary care.

Billing Focus: Use fourth-character specificity to distinguish between predominant B-cell abnormalities versus immunoregulatory T-cell disorders.

Explicitly link secondary manifestations such as bronchiectasis or interstitial lung disease to the primary CVID diagnosis.

Example: The patient has common variable immunodeficiency (D83.9) complicated by non-cystic fibrosis bronchiectasis (J47.9) in the right lower lobe, confirmed by high-resolution CT. This manifestation indicates a higher severity of the underlying immune deficiency. Billing includes both the primary immunodeficiency and the chronic respiratory complication to accurately reflect clinical resource intensity.

Billing Focus: Capture all secondary manifestations including bronchiectasis, lymphadenopathy, or enteropathy as additional codes.

Identify and document associated autoimmune disorders as they are frequent in CVID patients.

Example: Diagnosis of common variable immunodeficiency with autoantibodies to B-cells (D83.2) alongside concurrent immune thrombocytopenia (D69.3). Management requires balancing immunoglobulin replacement with immunosuppressive therapy for ITP. Documentation of the specific autoimmune component is necessary for appropriate risk stratification.

Billing Focus: Ensure the presence of autoantibodies or specific autoimmune phenotypes is documented to support D83.1 or D83.2.

Report the mode of administration and response for immunoglobulin replacement therapy.

Example: Patient with CVID (D83.9) currently maintained on subcutaneous immunoglobulin (SCIG) 20 percent solution weekly. Trough levels are stable at 850 mg/dL, with a significant reduction in the frequency of sinopulmonary infections over the last 12 months. This status code (Z79.899) supports the ongoing medical necessity for high-cost biologic therapy.

Billing Focus: Include Z79.899 for long-term use of other medications to document the chronic maintenance of immunoglobulin therapy.

Detail the lack of vaccine response or specific antibody deficiency findings from laboratory testing.

Example: Common variable immunodeficiency (D83.9) confirmed by a failure to produce protective antibody titers following the 23-valent pneumococcal polysaccharide vaccine. IgG levels are consistently below 400 mg/dL. Documentation of the impaired vaccine challenge is critical for validating the medical necessity of the diagnosis and subsequent IVIG authorization.

Billing Focus: Document the specific failure of antibody response to antigens to support the clinical validity of the ICD-10 code.

Relevant CPT Codes

Related Diagnoses

Hierarchy