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CVID- Major features

CVID- Major features. Recurrent pyogenic infections, with onset at any age Increased incidence of autoimmune disease Total immunoglobulin level < 300 mg/dL ,with IgG level < 250 mg/dL B cell numbers usually normal. CVID. Commonest symptomatic primary antibody deficiency

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CVID- Major features

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  1. CVID- Major features • Recurrent pyogenic infections, with onset at any age • Increased incidence of autoimmune disease • Total immunoglobulin level < 300 mg/dL ,with IgG level < 250 mg/dL • B cell numbers usually normal

  2. CVID • Commonest symptomatic primary antibody deficiency • Heterogenous group of immunological disorders, decrease serum Ig and antibody response • Prevalence: 1/50,000 – 1/100,000 • Occur in any age • Sex: equal

  3. Etiology • B lymphocytes unable to differentiate into plasma cells • B cell defectT cell defectActivation defect

  4. B Cell Defect • CVID B cell : phenotypic characteristics of immature B cell • CVID B cell+ normal T cell / CVID T cell + normal B cell • CVID B cell + mitogen + soluble T cell factors • Lack of external stimuli -> maturation arrest at immature B cell

  5. T Cell Defect • 25-30% patients  CD4/CD8 ratio •  IL-2, IL-4, IL-5, INF-g • Decreased proliferation to mitogen

  6. Suppressor T Cell DefectOverabundance -> lymphokines suppressing immunoglobulin synthesis (g-INF) • Activation DefectCD40 ligand (gp39) mRNA and functional protein in 40% patients • Genetic AbnormalityHLA class III region , between C4B and C2 genes

  7. Clinical Manifestations • Infections • Autoimmunity • Gastrointestinal disease • Lymphoproliferative disorders • Granulomatous disease

  8. Infections • Recurrent bacterial infection caused by encapsulated bacteria, Streptococcus pneumoniae, Haemophilus influenzae, Staphylococcus aureus • Bordetella pertussis important in childhood • Frequent complication: chronic sinusitis and bronchiectasis • Fungus, mycbacteria, Pneumocystis carinii : rare • Enteroviral meningoencephalitis : echovirus-11

  9. Profile of Infections (%) Recurrent bronchitis, sinusitis, otitis 98 Pneumonia 76.6 Viral Hepatitis 6.6 H/O severe Herpes zoster 3.6 Giardia enteritis 3.2 Pneumocystis carini infection 2.8 Mycoplasma pneumonia 2.4 Chronic Mucocutaneous candidiasis, Salmonella diarrhea, Campylobacter enteritis, Osteomyelitis, Septic arthritis, Nocardia brain abscess, etc. Cunningham-Rundes and Bodian (Clin Immunol 1999; 92:34-48)

  10. Autoimmunity (n=248) AITP 15 AIHA 12RA 5JRA 4Anti-IgA 7SS 2PBC 3Pernicious anemia 3Hyperthyroid disease 2SLE 2Vasculitis 3 autoimmune diseases 56

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