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.
Pediatric autoimmune liver disease and extra-hepatic immune-mediated comorbidities / G. Paolella, M. Farallo, I. Degrassi, C. Agostoni, C. Amoruso, F. Nuti, G. Nebbia. - In: DIGESTIVE AND LIVER DISEASE. - ISSN 1590-8658. - 51:2(2019 Feb), pp. 281-285.
|Titolo:||Pediatric autoimmune liver disease and extra-hepatic immune-mediated comorbidities|
PAOLELLA, GIULIA (Primo)
|Parole Chiave:||Autoimmune hepatitis; Autoimmune sclerosing cholangitis; Children; Extra-hepatic immune-mediated disorders; Hepatology; Gastroenterology|
|Settore Scientifico Disciplinare:||Settore MED/38 - Pediatria Generale e Specialistica|
|Data di pubblicazione:||feb-2019|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1016/j.dld.2018.07.033|
|Appare nelle tipologie:||01 - Articolo su periodico|
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