Background and aim: No comprehensive and prospective data are available for concurrent extrahepatic autoimmune disorders (CEAIDs) in children with autoimmune liver disease (AILD). The aim of this study was to evaluate CEAIDs in AILD and their effect on AILD outcome.
Patients and methods: Enrolled AILD and CEAIDs children were diagnosed on the basis of simplified and standard diagnostic criteria, respectively. The clinicopathological profile, treatment response, and outcome were compared between AILD with CEAIDs (group A) and AILD without CEAIDs (group B).
Results: In 62 AILD children, CEAIDs were found in 42% (n=26) [vitiligo (42%), celiac disease (CD) (15%), potential CD (15%), autoimmune hemolytic anemia (AIHA) (15%)]. CEAIDs were asymptomatic in 75%. Single CEAID was found in 81% (21/26) and multiple CEAID was found in 19% (5/26). Significantly less biochemical remission (46.1 vs. 74.2%, P=0.03), more treatment failure (23 vs. 3.2%, P=0.04), and higher mortality (15.3 vs. 3.2%, P=0.04) were encountered in group A compared with group B. On multivariate analysis (n=57), less biochemical remission in vitiligo (P=0.04); more treatment failure in AIHA (P=0.004) and vitiligo (P=0.04); and high mortality in AIHA (P=0.02) subgroups were reported. CD treatment has good impact on AILD outcome. All cases of diabetes mellitus in AILD were steroid-induced rather than because of autoimmunity (absence of antibody against tyrosine phosphatase and glutamic acid decarboxylase and elevated C-peptide).
Conclusion: All AILD children should be screened for CEAIDs as the majority are asymptomatic. The AILD outcome was favorable in CD, but poor in vitiligo and AIHA. We suggest the incorporation of CEAIDs in a pediatric AILD scoring system.