Autoimmunity and infection in common variable immunodeficiency (CVID).

Autoimmune phenotype in patients with common variable immunodeficiency.

BACKGROUND AND OBJECTIVE Autoimmune disorders occur with a higher incidence in common variable immunodeficiency (CVID) patients than in the general population. To describe the clinical features of the autoimmune phenotype in patients with CVID. METHODS The hospital records of all diagnosed CVID patients referred to the Children's Medical Center Hospital in Tehran, Iran between 2000 and 2010 w...

Pathogenesis of autoimmunity in common variable immunodeficiency

Common variable immunodeficiency (CVID) presents in up to 25% of patients with autoimmune (AI) manifestations. Given the frequency and early onset in some patients with CVID, AI dysregulation seems to be an integral part of the immunodeficiency. Antibody-mediated AI cytopenias, most often affecting erythrocytes and platelets make up over 50% of these patients. This seems to be distinct from mai...

Pediatric Systemic Lupus Erythematosus (SLE) As an Onset of Common Variable Immunodeficiency (CVID): The Double Link between Immunodeficiency and Autoimmunity

Recurrent, unusual and complicated infections are the hallmark of immunodeficiency. Primary Immunodeficiency (PID) is caused by a genetic defect impairing the immune response. Common Variable Immunodeficiency (CVID) is classified as a primary immunodeficiency with prevalent antibody defect, manifesting with respiratory and gastrointestinal infections. However, CVID is characterized by an increa...

Co-existence of Common Variable Immunodeficiency (CVID) with Idiopathic Thrombocytopenic purpura (ITP)

Thrombocytopenia in common variable immunodeficiency patients – clinical course, management, and effect of immunoglobulins

Common variable immunodeficiency (CVID) is a primary immunodeficiency of humoral immunity with heterogeneous clinical features. Diagnosis of CVID is based on hypogammaglobulinaemia, low production of specific antibodies, and disorders of cellular immunity. The standard therapy includes replacement of specific antibodies with human immunoglobulin, prophylaxis, and symptomatic therapy of infectio...