https://www.ncbi.nlm.nih.gov/pubmed/30166220

Dig Liver Dis. 2018 Aug 3. pii: S1590-8658(18)30870-3. doi: 10.1016/j.dld.2018.07.033. [Epub ahead of print]

Pediatric autoimmune liver disease and extra-hepatic immune-mediated comorbidities.
Paolella G1, Farallo M1, Degrassi I1, Agostoni C1, Amoruso C1, Nuti F1, Nebbia G2.

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.