Management of refractory cholestatic pruritus: An exceptional case

Abstract

Cholestatic pruritus is multifactorial and devastating. We report the case of a woman with primary biliary cholangitis overlapping with autoimmune hepatitis, a severe cholestatic profile, and intolerance to previous treatments. Magnetic resonance cholangiography revealed primary sclerosing cholangitis, and endoscopic retrograde cholangiopancreatography showed a stenosis treated with stent placement; despite biliary decompression, pruritus persisted. Therapeutic plasma exchange was indicated, and seven sessions were completed. During the cycle, there was a marked improvement in pruritus and recovery of sleep, with a sharp decrease in alkaline phosphatase and a sustained reduction in alanine aminotransferase and aspartate aminotransferase, while total and direct bilirubin showed a later and fluctuating decrease, gamma-glutamyltransferase stabilized at lower values. Subsequent removal of the stent was performed without complications and with pain control. The clinical and analytical course was consistent with a multicomponent pruritogenic syndrome involving bile acids with activation of pruriceptive receptors, via lysophosphatidic acid-autotaxin pathway and increased mu-opioid receptor tone, in which therapeutic plasma exchange acts as a bridge therapy by removing pruritogens and generating a gradient from the tissues to the plasma.