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Autoimmune liver diseases and inflammatory bowel diseases in children: current issues and future perspectives
Sabrina Cardile1, Tommaso Alterio2, Manila Candusso1
1a Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
Insights
Inflammatory bowel diseases (IBD) can affect the liver, with primary sclerosing cholangitis (PSC) being common in children. Further research is needed for better diagnosis and management of these complex cases.
Area of Science:
- Gastroenterology and Hepatology
- Pediatric Inflammatory Bowel Disease
- Hepatobiliary Manifestations
Background:
- Inflammatory bowel diseases (IBD) are chronic gut inflammation disorders with potential systemic effects.
- Extraintestinal manifestations occur in over 20% of pediatric IBD cases.
- Hepatobiliary involvement is frequent, with primary sclerosing cholangitis (PSC) affecting 6.4-7.8% of pediatric IBD patients.
Purpose of the Study:
- To review recent literature on hepatobiliary involvement in IBD patients.
- To focus on primary sclerosing cholangitis (PSC) in pediatric IBD.
- To provide updated information for diagnosis and management.
Main Methods:
- Literature review of recent studies on IBD and hepatobiliary manifestations.
- Analysis of data concerning primary sclerosing cholangitis (PSC) in pediatric IBD.
- Synthesis of current knowledge on diagnosis and management strategies.
Main Results:
- Hepatobiliary system involvement is a significant complication in IBD, particularly PSC in children.
- The pathogenesis of IBD-PSC linkage is multifactorial and not fully understood.
- Current therapeutic strategies for IBD-PSC are not standardized, with ongoing research into new medications.
Conclusions:
- The IBD-PSC association presents a unique model for studying disease mechanisms.
- Multidisciplinary approaches are essential for managing these complex patients.
- Further research is crucial to elucidate pathogenesis and improve patient care.
Abstract:
Inflammatory bowel diseases (IBDs) represent a group of intestinal disorders with a chronic and relapsing inflammation of the gut, and with a potential risk of systemic involvement of other organs and systems. Over the pediatric age, an incidence higher than 20% of developing extraintestinal manifestation during follow-up has been reported. The liver and the biliary system are frequently involved, and primary sclerosing cholangitis (PSC) is the most predominant entity with an incidence rate of 6.4-7.8% in children. PSC recognizes a multifactorial pathogenesis, and so far a not fully known mechanism for this association. The peculiar phenotype and the distinct clinical course of patients with IBD and PSC-associated make this 'linkage' an attractive study model to better understand mechanisms underlying these diseases. Approaching to these patients is complex and multidisciplinary, and a unique therapeutic strategy has not been standardized yet. New medications are being studied; however, further studies are needed to fully understand the pathogenesis and to improve the care of these patients. The aim of this paper is to review the recent literature regarding hepatobiliary involvement in IBD patients, with particular attention to PSC, and to provide the latest information for a correct diagnosis and appropriate management.
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