title: Development of systemic sclerosis in patients with autoimmune hepatitis: an emerging overlap syndrome.
abstract: OBJECTIVE
  We described two case reports of AIH/SSc overlap syndrome and reviewed literatures regarding this issue.
  
  
  BACKGROUND
  AIH is a chronic hepatitis of unknown aetiology characterized by continuing hepatocellular necrosis and inflammation. AIH overlap syndromes have been reported with other autoimmune diseases.
  
  
  METHODS
  According to the classification criteria for SSc, we conducted a retrospective chart review of 35 cases with biopsy-proven AIH over the past 5 years at our institution. We reviewed the MEDLINE database using the appropriate key-words.
  
  
  RESULTS
  A chart review of 35 cases (M/F ratio 1:2, mean age 47.6±10.3 years) revealed nine patients (9/35, 25.7%) with CTD (four males and three females with a mean age of 45.1±8.4 years). All patients had ANA. Four patients were SSA/Ro positive UCTD (1/35, 2.85%), and six patients developed SLE (6/35, 17.1%). Only two female patients (2/35, 5.7%) with specific SSc AAb developed a systemic sclerosis. We described a patient with AIH who was diagnosed with diffuse systemic sclerosis-sine scleroderma with positive anti-centromere B and SSA/Ro52 KDa antibodies. We also reported a patient with AIH who was diagnosed limited SSc with contemporary presence of anti-centromere A and anti-RNA polymerase III antibody.
  
  
  CONCLUSIONS
  We suggest that SSc may be considered to be one of the manifestations associated with AIH. Patients with AIH may have an increased risk to develop SSc and should be followed, especially when Raynaud phenomenon was found.
authors.affiliation: Clinical investigation laboratory, Humanitas Clinical and Research Center, Via Alessandro Manzoni 56 20089 Rozzano (Miano) Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Assandri
authors.affiliation: Clinical investigation laboratory, Humanitas Clinical and Research Center, Via Alessandro Manzoni 56 20089 Rozzano (Miano) Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Monari
authors.affiliation: Clinical Laboratory, Diagnostics Department, Spedali Civili of Brescia, Piazzale Spedali Civili 1 - Brescia, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Montanelli
keywords: Autoimmune hepatitis; Autoimmune liver diseases; Systemic sclerosis