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Allergy and Immunology›Condition·Updated Jul 11, 2026·v1

Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency in adults, characterized by hypogammaglobulinemia, defective antibody responses, and recurrent sinopulmonary infections. Non-infectious complications including autoimmune cytopenias, lymphoproliferation, GLILD, and malignancy now dominate morbidity and mortality. Early immunoglobulin replacement reduces infections, but management of complications requires targeted immunosuppression and close surveillance.

Moderate Evidence235 references·7,593 words·31 min read·v1
Common Variable ImmunodeficiencyCVIDPrimary ImmunodeficiencyHypogammaglobulinemiaRecurrent InfectionsAutoimmune CytopeniasGLILDNFKB1CTLA4Immunoglobulin Replacement

Quick Reference

RxDrug of choiceImmunoglobulin replacement therapy (IVIG 400-600 mg/kg q3-4wk or SCIG 100-200 mg/kg weekly) titrated to individual trough.
AltAlternativesAntimicrobial prophylaxis (TMP-SMX DS daily or azithromycin 250 mg TIW); for non-infectious complications: prednisone ≥0.3 mg/kg/day, rituximab 150-375 mg/m², abatacept (CTLA-4 insufficiency), sirolimus (LRBA deficiency), omalizumab (CSU), PD-1 inhibitors (malignancy).
⊘AvoidLive vaccines; IgA-containing IVIG in patients with anti-IgA antibodies; non-dihydropyridine CCBs (diltiazem, verapamil) in CVID with cardiac involvement.
DxTest of choiceSerum immunoglobulins (IgG, IgA, IgM, IgE) plus vaccine response testing (pneumococcal polysaccharide + protein antigens) plus B-cell immunophenotyping (switched memory B cells, CD21low B cells).
ScKey scoreEUROclass classification (smB <2% = high risk; CD21low >10% = lymphoproliferation) and clinical phenotype (infection-only vs complicated CVID).
→When to referRefer to immunology at diagnosis; consider specialized center for complicated CVID (non-infectious complications, monogenic defect, refractory disease, need for HSCT or targeted biologic).
CVID is the most common primary immunodeficiency in adults, driven by humoral and T-cell defects. Early immunoglobulin replacement reduces infections, but non-infectious complications (autoimmune cytopenias, lymphoproliferation, GLILD, malignancy) determine prognosis and require targeted immunosuppression. Monogenic causes guide therapy, and systematic surveillance improves outcomes.
Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency in adults, characterized by low serum immunoglobulins (IgG and IgA and/or IgM) and impaired specific antibody responses. The disease manifests with recurrent sinopulmonary infections, autoimmune cytopenias, lymphoproliferation, enteropathy, and increased malignancy risk, complications that now dominate morbidity and mortality. Diagnostic delays of 5-8 years are common. Early immunoglobulin replacement therapy reduces infection frequency and improves survival; effective management requires parallel strategies for infectious prophylaxis and targeted immunosuppression for non-infectious complications. This overview synthesizes the current paradigm for evaluation and management.

Overview and Recommendations

Background

  • •CVID is the most common symptomatic primary immunodeficiency in adults, with an estimated prevalence of 1 in 500 to 1 in 500,000. It is defined by low serum IgG and low IgA (with or without low IgM) and defective specific antibody production, leading to recurrent infections and immune dysregulation that spans B- and T-cell compartments.
  • •The classic presentation involves recurrent sinopulmonary infections (pneumonia as first manifestation in 32% of patients), but non-infectious complications, autoimmune cytopenias (ITP, AIHA, Evans syndrome), lymphoproliferation (splenomegaly, lymphadenopathy), enteropathy, granulomatous-lymphocytic interstitial lung disease (GLILD), and malignancy, now drive most morbidity. The presence of even one non-infectious complication confers an 11-fold increase in mortality compared with infection-only disease.
  • •Pathophysiology centers on a fundamental B-cell maturational defect (reduced class-switched memory B cells) compounded by T-cell dysregulation: follicular helper T-cell expansion, regulatory T-cell deficiency, and chronic innate immune activation driven by microbial translocation. IgA deficiency with impaired anti-LPS antibodies permits endotoxemia, which triggers a BTK-dependent proinflammatory cytokine cascade (TNF-α, IL-6, IFN-γ, CXCL9, CXCL10) in genetically susceptible individuals.
  • •Monogenic causes are identified in 20-30% of patients, most commonly heterozygous loss-of-function variants in (4% of Europeans), haploinsufficiency, deficiency, and defects in , , , and . Identification of a specific genetic defect can guide targeted therapy (e.g., abatacept for CTLA-4 insufficiency, sirolimus for LRBA deficiency) and consideration of hematopoietic stem cell transplantation.
  • •Diagnostic criteria per ESID/ICON require onset after age 2 years, low IgG and low IgA (and/or low IgM), impaired vaccine responses to pneumococcal polysaccharide and protein antigens, and exclusion of secondary causes of hypogammaglobulinemia. Patients are clinically stratified into infection-only (CVIDio) and complicated CVID (CVIDc), the latter encompassing autoimmune, lymphoproliferative, enteropathic, and malignant phenotypes, to guide prognosis and surveillance intensity.

Evaluation

  • •Suspect CVID in any adult with recurrent sinopulmonary infections (≥2 pneumonias in 1 year, chronic sinusitis, otitis media), especially when accompanied by unexplained autoimmune cytopenia, splenomegaly, lymphadenopathy, chronic diarrhea, or bronchiectasis.
  • •Ask about age at first serious infection, frequency of antibiotic courses, history of pneumonia, bronchiectasis, autoimmune disease (ITP, AIHA, Evans syndrome), family history of immunodeficiency, and prior immunoglobulin measurements. Diagnostic delay averages 5-8 years, so maintain a low threshold.
  • •Examine for signs of chronic lung disease (crackles, clubbing), lymphadenopathy, splenomegaly, mucocutaneous candidiasis, and skin findings (vitiligo in 8.5%, eczema in 22.4%, purpura from thrombocytopenia).
  • •Order initial laboratory workup: complete blood count with differential, serum immunoglobulins (IgG, IgA, IgM, IgE), and serum protein electrophoresis with calculated globulin (CG <22 g/L has 80% sensitivity, 87% specificity for hypogammaglobulinemia). Low IgG and low IgA are required for diagnosis.
  • •Evaluate specific antibody responses by measuring pre- and 4-week post-vaccination titers to pneumococcal polysaccharide (Pneumovax) and protein antigens (tetanus, diphtheria). Failure to achieve a ≥2-fold rise or protective levels in <70% of serotypes defines the defect. If the patient is already on IVIG, use ELISPOT assay for antibody-secreting cells instead of serology.
  • •Perform B-cell immunophenotyping by flow cytometry: quantify switched memory B cells (CD27+IgD-), marked reduction (<2% of B cells) is a hallmark of CVID and predicts non-infectious complications. Expansion of CD21low B cells (>5% of B cells) correlates with GLILD and splenomegaly.
  • •Assess T-cell subsets: low naive CD4+ T cells (<20% of total CD4+) defines late-onset combined immunodeficiency (LOCID), which independently predicts splenomegaly, lymphadenopathy, interstitial lung disease, lymphoma, and shortened survival.
  • •Consider serum B-cell maturation antigen (sBCMA) as a novel biomarker: <15 ng/mL has 97% positive predictive value for CVID or X-linked agammaglobulinemia; ≥25 ng/mL has 88% negative predictive value.
  • •Genetic testing (targeted gene panel or whole exome) is indicated for patients with non-infectious complications dominating the phenotype, family history of immunodeficiency, or severe/atypical infections. Identification of a monogenic cause (NFKB1, CTLA4, LRBA, etc.) can direct targeted therapy and cascade screening of relatives.
  • •In patients with atopic symptoms but negative skin prick testing or serum specific IgE, consider bronchial challenge to confirm allergic asthma, as standard allergy testing is often falsely negative in CVID (only 9.7% have atopy by skin test despite suggestive histories).
  • •Rule out secondary causes of hypogammaglobulinemia: medications (rituximab, anticonvulsants, sulfasalazine), protein-losing enteropathy, nephrotic syndrome, lymphoproliferative diseases (CLL, thymoma/Good syndrome), and chronic infections (HIV, EBV, CMV).
  • •Perform baseline screening for established complications: high-resolution chest CT (HRCT) to assess for bronchiectasis and GLILD, pulmonary function tests including FVC and DLCO, and upper endoscopy with standardized biopsies to screen for atrophic gastritis and intestinal metaplasia (found in 34% of CVID patients) if gastrointestinal symptoms are present.
  • •Refer to an immunologist for confirmation and initiation of immunoglobulin replacement therapy; coordinate a multidisciplinary approach including pulmonology, gastroenterology, and hematology for complicated cases.

Management

  • •Initiate immunoglobulin replacement therapy (IgRT) as soon as the diagnosis is confirmed. Start 400-600 mg/kg every 3-4 weeks or 100-200 mg/kg weekly; titrate the dose to maintain a trough IgG >700-800 mg/dL, though the ideal level should be individualized based on breakthrough infection frequency.
  • •SCIG is increasingly preferred for its flexibility, fewer systemic adverse effects, suitability for home administration, and improved quality of life. IVIG remains appropriate for patients who prefer center-based therapy or have adherence concerns.
  • •For breakthrough sinopulmonary infections despite adequate trough IgG levels, add antimicrobial prophylaxis: one double-strength tablet daily or 250 mg three times weekly. Tailor choice based on sputum culture results when possible.
  • •Manage GLILD with high-dose corticosteroids: initiate at ≥0.3 mg/kg daily (or equivalent). This regimen improves HRCT scores and forced vital capacity, achieving durable remission in approximately 42% of patients. Low-dose maintenance (<0.3 mg/kg) provides no added benefit, taper to the lowest effective dose over 4-8 weeks.
  • •For steroid-refractory or relapsing GLILD, use -based therapy. Low-dose rituximab 150 mg/m² weekly may achieve remission with fewer infections; standard-dose rituximab 375 mg/m² every 3 weeks is an alternative but carries a higher risk of opportunistic infections. Combination with azathioprine (dose per protocol) has shown benefit in case series.
  • •For autoimmune cytopenias (ITP, AIHA, Evans syndrome), first-line treatment is high-dose corticosteroids (e.g., pulse therapy). For refractory cases, consider rituximab, azathioprine, or other steroid-sparing immunosuppressants. Maintain IgG trough >7 g/L to reduce autoimmune thrombocytopenia episodes.
  • •In monogenic CVID due to haploinsufficiency, administer abatacept (CTLA-4 fusion protein) as targeted therapy. Hematopoietic stem cell transplantation (HSCT) can achieve sustained remission in 72% of CTLA-4 mutation carriers and is also an option for severe deficiency or other monogenic defects with refractory immune dysregulation.
  • •For LRBA deficiency, (mTOR inhibitor) has demonstrated efficacy in reducing lymphoproliferation and autoimmunity; consider HSCT if disease is severe and progressive.
  • •For refractory chronic spontaneous urticaria in CVID, can induce complete remission after the first injection and is well tolerated over 12 months.
  • •For malignancy arising in CVID (e.g., lung adenocarcinoma, lymphoma), (nivolumab, pembrolizumab) have been used successfully, achieving partial response with progression-free survival >15 months. Do not automatically exclude CVID patients from checkpoint inhibitor trials.
  • •Vaccination: administer inactivated influenza vaccine annually and mRNA vaccine series with boosters as recommended. Humoral responses develop in 52-83% of CVID patients but wane rapidly; T-cell responses are more durable and correlate with protection against severe disease. Live vaccines are contraindicated.
  • •Monitor for complications systematically: perform annual pulmonary function tests; repeat HRCT every 1-2 years if GLILD is present (consider MRI as radiation-sparing alternative); screen for gastric cancer with baseline upper endoscopy and standardized biopsies at diagnosis, then every 3-5 years (especially in smokers or with H. pylori infection).
  • •Intubation criteria for acute respiratory failure: FVC <20 mL/kg ideal body weight, PaO2/FiO2 ratio <150 mm Hg on non-rebreather, hypercapnia (PaCO2 >50 mm Hg with pH <7.30), or signs of impending fatigue (accessory muscle use, respiratory rate >35 breaths/min, abdominal paradox).
  • •Provide standard VTE prophylaxis for hospitalized CVID patients: 40 mg SC daily or 5000 U SC twice daily, unless bleeding risk from severe thrombocytopenia or active bleeding.
  • •Refer patients with complicated CVID (non-infectious complications, monogenic defect, refractory disease) to specialized immunology/transplant centers for consideration of targeted biologic therapies or HSCT.
  • •In pregnancy, continue IgRT to maintain trough IgG >500-700 mg/dL; IVIG is safe with no moderate/severe adverse effects. Coordinate multidisciplinary care with maternal-fetal medicine, immunology, and neonatology. Breastfeeding is encouraged as colostrum contains protective antibodies against enteropathogenic E. coli.
  • •In elderly patients, individualize IgG trough targets to balance infection prevention against volume overload; SCIG may be preferred to avoid hemodynamic stress of IVIG. Monitor for comorbidities (chronic lung disease, diabetes, hypertension) that complicate management.
  • •Avoid non-dihydropyridine calcium channel blockers (diltiazem, verapamil) as they exacerbate heart failure in patients with cardiac involvement. Avoid IgA-containing immunoglobulin products in patients with known anti-IgA antibodies at risk of anaphylaxis.
  • •Monitor for iatrogenic complications: corticosteroids increase opportunistic infection risk (2 cases in one series); rituximab may worsen humoral deficiency (compensated by IgRT); checkpoint inhibitors can trigger immune-related adverse events. Adjust immunosuppression in collaboration with the primary immunologist.

Board Review — High Yield

  • •Low IgG + IgA + impaired vaccine responses, Diagnostic triad for CVID; require onset after age 2 and exclusion of secondary causes.
  • •EUROclass smB- group, Switched memory B cells <2% of total B cells predicts splenomegaly, granulomatous disease, and lymphoma.
  • •CVIDc (complicated CVID), Presence of any non-infectious complication (cytopenias, lymphoproliferation, enteropathy) increases mortality 11-fold.
  • •NFKB1 haploinsufficiency, Most common monogenic cause (4% of Europeans); causes progressive B-cell defect, autoimmunity, and bronchiectasis.
  • •GLILD, Granulomatous-lymphocytic interstitial lung disease; diagnosed by HRCT + biopsy; treated with high-dose corticosteroids (≥0.3 mg/kg prednisone) ± rituximab.
  • •IVIG vs SCIG, Both first-line; SCIG has fewer systemic reactions and enables home therapy; target trough IgG >700-800 mg/dL.
  • •CTLA-4 insufficiency, Responds to abatacept; HSCT can be curative.
  • •Vaccination in CVID, Humoral responses blunted (52-83% seroconversion to COVID-19 mRNA vaccine, rapid waning); T-cell responses more durable; live vaccines contraindicated.
  • •Diagnostic delay, Averages 5-8 years; maintain high index of suspicion in adults with recurrent infections + autoimmunity or lymphoproliferation.
  • •Anaphylaxis risk, FCGR2A gain-of-function variant predisposes to IVIG reactions; switch to SCIG or use IgA-depleted products.

Deep Dive — Evidence Details

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