title: Effectiveness and safety of ciclosporin as therapy for autoimmune diseases of the liver in children and adolescents.
abstract: BACKGROUND
  Conventional treatment for autoimmune hepatitis results in a significant percentage of failures and several, poorly tolerated, side-effects. Therapy for autoimmune cholangitis and giant cell hepatitis associated with autoimmune haemolysis is poorly documented. Ciclosporin is a promising treatment for all of these diseases.
  
  
  METHODS
  We reviewed the records of 12 patients treated in our unit between 1987 and 2001. Eight had autoimmune hepatitis, two had autoimmune cholangitis and two had giant cell hepatitis. Indications for ciclosporin were treatment failure (four patients) and contraindications to/refusal of steroids (eight patients). Ciclosporin was administered in five untreated cases and in seven patients during relapse. The mean duration of ciclosporin administration was 35.6 months (8-89 months). The median follow-up was 6.5 years (1.5-15 years).
  
  
  RESULTS
  All patients achieved complete remission in a median period of 4.5 weeks (2-12 weeks). No treatment withdrawal due to side-effects occurred. Three patients required a combination of ciclosporin with conventional treatment due to severe liver function impairment. Tolerance to ciclosporin was excellent. A 20% transient elevation of serum creatinine occurred in one case, gingival hypertrophy in two and moderate hypertrichosis in two.
  
  
  CONCLUSIONS
  Ciclosporin may be considered as a safe treatment for all autoimmune liver diseases and as an effective alternative for front-line therapy.
authors.affiliation: Gastroenterologia ed Epatologia Pediatrica, Dipartimento di Medicina della Procreazione e della Età Evolutiva, Università di Pisa, Pisa, Italy. g.maggiore@clp.med.unipi.it
authors.forename: M
authors.initials: M
authors.lastname: Sciveres
authors.forename: S
authors.initials: S
authors.lastname: Caprai
authors.forename: G
authors.initials: G
authors.lastname: Palla
authors.forename: C
authors.initials: C
authors.lastname: Ughi
authors.forename: G
authors.initials: G
authors.lastname: Maggiore
mesh_terms: D001327:Autoimmune Diseases / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008107:Liver Diseases / Q000188:drug therapy*; D008297:Male; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine