title: A case of severe recurrent hepatitis with common variable immunodeficiency.
abstract: Severe hepatitis with an indistinct etiology manifested in a 16-year-old boy who had no particular history. The histological features of the liver and clinical course of the patient were similar to those of patients with autoimmune hepatitis characterized by interface hepatitis and severe lobular inflammation of the liver and recurrent exacerbations of hepatitis. We administered intravenous glycyrrhizin preparation daily or three times a week combined with the oral administration of ursodeoxycholic acid daily throughout the term after the initial onset of disease for the control of disease activity. The normalization of the concentration of alanine aminotransferase in serum was achieved in response to the therapy during the course. The serum concentration of immunoglobulins of the patient gradually decreased from the onset of the disease to an unacceptable level without globulin preparation during the following period of 17 months. Immunological tests revealed impairment of immunoglobulin production bythe B cell population of the patient, which led to the diagnosis of the patient as common variable immunodeficiency (CVID). The patient, with improved liver histology after 27 months from the onset of disease, benefited from the current combination therapy without severe infection through the avoidance of overimmunosuppression. CVID is defined as a heterogeneous syndrome characterized by various degrees of hypogammaglobulinemia without any specific predisposing causes, frequently associated with autoimmunity. Diagnostic criteria and therapeutic options of persistent hepatitis with CVID are to be established, as discussed in the current report.
authors.affiliation: Department of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Fukushima
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ueno
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Kanegane
authors.forename: Yoko
authors.initials: Y
authors.lastname: Yamagiwa
authors.forename: Jun
authors.initials: J
authors.lastname: Inoue
authors.forename: Osamu
authors.initials: O
authors.lastname: Kido
authors.forename: Futoshi
authors.initials: F
authors.lastname: Nagasaki
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kogure
authors.forename: Eiji
authors.initials: E
authors.lastname: Kakazu
authors.forename: Yu
authors.initials: Y
authors.lastname: Nakagome
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Matsuda
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Obara
authors.forename: Osamu
authors.initials: O
authors.lastname: Kimura
authors.forename: Tooru
authors.initials: T
authors.lastname: Shimosegawa