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Concurrent extrahepatic autoimmune disorders: unexplored dimension of autoimmune liver disease in children
Vikas Jain1, Surender K Yachha1, Eesh Bhatia2
1Departments of Pediatric Gastroenterology.
Insights
Children with autoimmune liver disease (AILD) often have concurrent extrahepatic autoimmune disorders (CEAIDs), frequently asymptomatic. Screening for CEAIDs in AILD is recommended, as their presence impacts AILD outcomes, particularly in vitiligo and autoimmune hemolytic anemia.
Area of Science:
- Pediatric Gastroenterology
- Autoimmunology
- Hepatology
Background:
- Limited data exists on concurrent extrahepatic autoimmune disorders (CEAIDs) in pediatric autoimmune liver disease (AILD).
- Understanding CEAIDs in AILD is crucial for managing patient outcomes.
Purpose of the Study:
- To evaluate the prevalence of CEAIDs in children with AILD.
- To assess the impact of CEAIDs on the outcomes of pediatric AILD.
Main Methods:
- Prospective study of 62 children diagnosed with AILD.
- Comparison of clinicopathological profiles, treatment response, and outcomes between AILD with and without CEAIDs.
- Diagnosis of AILD and CEAIDs based on established criteria.
Main Results:
- 42% of AILD patients had CEAIDs, most commonly vitiligo and celiac disease (CD).
- CEAIDs were asymptomatic in 75% of affected children.
- AILD with CEAIDs showed significantly lower biochemical remission rates, higher treatment failure, and increased mortality compared to AILD without CEAIDs.
- Specific CEAIDs like vitiligo and autoimmune hemolytic anemia (AIHA) were associated with poorer AILD outcomes, while CD showed a favorable impact.
Conclusions:
- Routine screening for CEAIDs in pediatric AILD is recommended due to high prevalence and asymptomatic nature.
- The presence of certain CEAIDs (vitiligo, AIHA) negatively impacts AILD prognosis.
- Incorporating CEAIDs into pediatric AILD scoring systems is suggested for improved management.
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.
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