Successful treatment with cyclosporine A of HCV-driven chronic liver disease mimicking autoimmune hepatitis in a patient with common variable immunodeficiency.
Common variable immunodeficiency (CVID) is the commonest primary immunodeficiency disease characterized by defective antibody production and various degrees of T cell numbers abnormality or impaired proliferation to mitogens. Clinical features include recurrent bacterial sinopulmonary and gastrointestinal infections. Autoimmunity is very common in CVID, occurring in approximately 25% of the patients particularly with autoimmune thrombocytopenia, hemolytic anemia, inflammatory bowel disease, and rheumatoid arthritis. Persistent antigen stimulation, secondary to a defective eradication of pathogens followed by a compensatory exaggerated chronic inflammatory response, is the primary cause leading to autoimmunity. Here we describe a girl with CVID in whom a chronic liver disease mimicking autoimmune hepatitis developed after hepatitis C virus infection. The immunosuppressive treatment with cyclosporine A proved effective in reversing liver disease.
Martire
Baldassarre
B
Dipartimento di Biomedicina dell'Età Evolutiva, University of Bari, Italy.
Gentile
Antonia
A
Francavilla
Ruggiero
R
De Santis
Attilio
A
De Mattia
Domenico
D
D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications*; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D019698:Hepatitis C, Chronic / Q000150:complications; D019693:Hepatitis, Autoimmune / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008099:Liver / Q000473:pathology
D007166:Immunosuppressive Agents; D016572:Cyclosporine