Abstract

RIOS, Marta et al. Autoimmune liver disease in children and adolescents: difficulties on diagnosis. J Port Gastrenterol. [online]. 2012, vol.19, n.5, pp.229-240. ISSN 0872-8178.

Introduction: Autoimmune liver disease (AILD) may involve predominantly the hepatocytes or the intra/extra-hepatic bile duct epithelium, or both structures, naming respectively, autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and overlap syndrome (OS). Aim: To identify the diagnostic difficulties of AILD in a paediatric population. Methods: Retrospective study of the cases followed in a pediatric hospital in the last 19 years. Results: Included 20 patients (10 M, 10F): AIH-10, PSC-7, OS-3. Median age at the time of onset of symptoms was 9,0 years and at the date of diagnosis was 11,5 years. Of the 10 patients with AIH, 3 had a pre-treatment score of probable diagnosis, and all had a post-treatment score of definitive diagnosis. In 1 patient the presence of dsDNA forced the differential diagnosis with lupus. Of the 7 patients with PSC, only 2 had abnormalities of the biliary tract on imaging, and 4 histological lesions in the ducts. Three patients had diagnostic criteria for OS, 1 with sequencial presentation. Conclusions: The major diagnostic difficulties were encountered in patients with PSC in early stage, and in cases of OS, especially the one of sequential presentation. Also noteworthy are the difficulties of the differential diagnosis between HAI as an independent entity and other multisystem autoimmune diseases with liver involvement, such as systemic lupus erythematosus.

Keywords : Autoimmune hepatitis; Primary sclerosing cholangitis; Overlap syndrome; Child.

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