Pediatric autoimmune liver disease and extra-hepatic immune-mediated comorbidities. Issue 2 (February 2019)

• Record Type: Journal Article
• Authors: Paolella, Giulia; Farallo, Marcello; Degrassi, Irene; Agostoni, Carlo; Amoruso, Chiara; Nuti, Federica; Nebbia, Gabriella
• Is Part Of: Digestive and liver disease. Volume 51:Issue 2(2019)
• Page Start: 281
• Page End: 285
• Publication Date: 2019-02
• Subjects: Autoimmune hepatitis -- Autoimmune sclerosing cholangitis -- Children -- Extra-hepatic immune-mediated disorders; Digestive organs -- Diseases -- Periodicals; Liver -- Diseases -- Periodicals; 616.33005
• DOI: 10.1016/j.dld.2018.07.033
• Languages: English
• ISSNs: 1590-8658

Abstract

Abstract: Background: Autoimmune liver disease (AILD) includes autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC). AILD is often associated with other extra-hepatic immune-mediated disorders (EDs), but there are few pediatric studies available to date. In this study we evaluated the association between AILD and EDs in our pediatric series. Methods: In this single centre retrospective study 48 patients (39 AIH and 9 ASC children) were evaluated. Thirty-six children were primarily referred to our Centre for liver disease suspicion, while the remaining twelve had a previous diagnosis of EDs. All the patients were screened for various EDs at AILD diagnosis and yearly during the follow-up. Results: Mean duration of follow-up was 9 years and 1 month. Twenty-two (46%) patients had a diagnosis of EDs. Ulcerative colitis (UC) was the most frequent EDs (9 patients), followed by autoimmune thyroid disease (5 patients) and celiac disease (5 patients). In 7 out of 9 UC patients, ASC was present. Conclusions: Our study showed a high association (46%) between AILD and EDs. In particular, in 8 out of 9 ASC patients UC was diagnosed (p-value 0.007). It is important to look for EDs in AILD children and, conversely, AILD in EDs children with abnormal liver function tests.