Cholestatic Pruritus in Children: Conventional Therapies and Beyond

SIMPLE SUMMARY: Pediatric patients with liver disease commonly experience itching, also known as pruritus. This symptom may seem to be a mere inconvenience, but can actually significantly affect the quality of life of these patients, including impairing their sleep and worsening their mental health. The cause of itching in liver disease is not fully known and is most likely caused by many factors. Unfortunately, this symptom can be incredibly difficult to treat and may ultimately require surgical interventions in certain cases that do not respond to medical therapy. Some medical therapies that are used to treat cholestatic pruritus in the adult population may be beneficial for pediatric patients suffering from this symptom. Ultimately, a better understanding of what causes itching in liver disease may provide valuable information for how to best treat this symptom. ABSTRACT: Pruritus in the setting of cholestatic liver disease is difficult to treat and occurs in patients ranging in age from infancy to adulthood. Likely multifactorial in etiology, this symptom often involves multimodal therapy targeting several pathways and mechanisms proposed in the underlying etiology of cholestatic pruritus. Many patients in both the pediatric and adult populations continue to experience unrelenting pruritus despite maximal conventional therapy. Options are further limited in treating pediatric patients due to sparse data regarding medication safety and efficacy in younger patients. Conventional therapies for the treatment of cholestatic pruritus in children include ursodeoxycholic acid, cholestyramine, hydroxyzine, and rifampin. Certain therapies are more routinely used in the adult populations but with limited data available for use in child and adolescent patients, including opioid antagonists and selective serotonin reuptake inhibitors. Recently, ileal bile acid transport inhibitors have been shown to alleviate pruritus in many children with Alagille syndrome and progressive familial intrahepatic cholestasis and is an additional therapy available for consideration for these patients. Ultimately, surgical options such as biliary diversion or liver transplantation are considered in specific circumstances when medical therapies have been exhausted and pruritus remains debilitating. While further investigation regarding underlying etiologies and effective therapies are needed to better understand itch pathogenesis and treatment in pediatric cholestasis, current considerations beyond conventional management include the use of opioid antagonists, selective serotonin reuptake inhibitors, ileal bile acid transport inhibitors, and surgical intervention.