Treatment of pruritus with Prometheus dialysis and absorption system in a patient with benign recurrent intrahepatic cholestasis

Abstract

Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive liver disorder characterized by recurrent episodes of jaundice and itching. Episodes of cholestasis last variously from 1 week to several months, may start at any age and usually resolve spontaneously. No effective treatment has been found as yet. We report a case of genetically proven BRIC in a male patient who developed three episodes of pruritus and jaundice at the age of 14, 16 and 19 years. During the third episode, he did not respond to pharmacological medical therapy, and fractionated plasma separation and absorption (FPSA, Prometheus) was performed to manage intractable pruritus. The treatment immediately alleviated pruritus, lowered serum bilirubin concentration and induced sustained remission in the 5-year follow up. FPSA seems to be a safe and effective way of treatment for BRIC in patients with severe pruritus and prolonged jaundice.

Successful treatment with 4‐phenylbutyrate in a patient with benign recurrent intrahepatic cholestasis type 2 refractory to biliary drainage and bilirubin absorption

“…We cannot completely exclude the possibility that the symptomatic relief in the patient was due to spontaneous resolution rather than to the 4PB therapy. However, the fact that the liver tests and itching score exacerbated immediately after the end of the 4PB therapy and that, in general, asymptomatic periods in BRIC patients last from months to years strongly suggests that 4PB therapy relieved the cholestatic episode in this patient with BRIC2, which was refractory to the medical and invasive therapy proposed by previous studies as optional treatments for BRIC …”

“…However, a reduction in the number and duration of the attacks is highly desirable, because intractable itching accompanied by cholestasis disrupts patients’ activities of daily living and their ability to sleep, making the patients disabled and depressed, and consequently decreases their quality of life and that of their families. There are case studies of medical therapy for cholestatic attacks in BRIC patients with rifampicin, cholestyramine and corticosteroids, and of invasive therapy with nasobiliary drainage (NBD) . However, all of these treatments are symptomatic therapy only, because of the lack of understanding of the molecular mechanism underlying intrahepatic cholestasis in BRIC, and inconsistent results have been reported for each treatment …”

Hepatitis C virus-associated pruritus: Etiopathogenesis and therapeutic strategies

“…Although the pruritogenic mediators and their receptors have been identified, there is no consensus regarding the mechanisms regulating the etiopathogenesis of pruritus. Apart from the suspected mediators and their origin, the onset of pruritus is closely associated with the development of intrahepatic cholestasis[ 31 ]. Although hepatic disorders such as chronic infection with viral hepatitis, primary biliary cirrhosis or primary sclerosing cholangitis are considered the main cause of intrahepatic cholestasis[ 32 , 33 ], the elevation of bile salts and μ-opioids levels in patients with intrahepatic cholestasis is largely associated with pruritus[ 34 ].…”

New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications

“…In addition to cholestyramine, phenobarbital, S-adenosylmethionine, opiate antagonists, UDCA, rifampicin, and serotonin antagonists and its reuptake inhibitors seem to be beneficial in managing refractory pruritus in patients with PFIC [ 77 , 91 , 92 ]. Furthermore, clinical and biochemical improvements have been observed in BRIC patients with intractable pruritus after using the molecular adsorbent recirculating system (MARS) and the Prometheus system, an extracorporeal liver support therapy based on fractionated plasma separation and adsorption [ 93 ].…”