Therapeutic plasma exchange for intractable pruritus secondary to primary sclerosing cholangitis

To the Editor: The 2013 American Society for Apheresis Guidelines do not include intractable pruritus caused by chronic cholestasis as a distinct indication for therapeutic plasma exchange (TPE) [1]. However, TPE has been shown to decrease bile salts and alleviate symptoms [2–4]. Some papers report that extracorporeal albumin dialysis using molecular adsorbents recirculating system (MARS VR ) has similar effects [5]. We describe our experience of successfully treating a patient with intractable pruritus secondary to primary sclerosing cholangitis (PSC) that was refractory to conventional therapy for many years. In 2002, a 58-year-old female presented to hepatology for management of PSC diagnosed 2 years prior. The patient complained of diffuse pruritus beginning in 1997, which was primarily treated only with ursodiol. Serial hepatic function tests performed between 11/2002 and 4/2012 demonstrated normal to mildly elevated values (Fig. 1). In 4/2012, she complained of worsening, constant, and severe pruritus refractory to ursodiol. Her worsening symptoms coincided with increased interval narrowing of the distal common bile duct and left intrahepatic duct strictures, as measured by magnetic resonance imaging. For 11 months, she was prescribed a variety of combinations of cholestyramine, naltrexone, ursodiol, sertraline, doxepin, rifampin, and hydroxyzine. A biliary stent was placed in 6/2012 with subsequent stricture dilatation and stent replacement occurring every 6–8 weeks. During this period, all hepatic function tests indicated progressive disease and persistent pruritus. In 3/2013, she was referred to the apheresis service for a trial of TPE. This initial series consisted of 7, every other day procedures, during which one plasma volume was replaced with 5% albumin. This series of procedures resolved the patient’s pruritic symptoms. However, her symptoms returned approximately 1 month after the trial, prompting a second series of 5 TPEs, which again alleviated her symptoms. Due to the recurrence of her symptoms and the success of the TPE trials, a dual lumen, permanent dialysis catheter was placed and the patient was started on maintenance therapy consisting of one TPE per week with 5% albumin as the replacement therapy. Twenty-four months later, she continues to have weekly TPE, in addition to taking cholestyramine (4g twice per day). Initially, she continued to have biliary stents replaced every 6–8 weeks while undergoing weekly TPE. However, due to her fear of the stent replacement procedure, she and her hepatologist decided to discontinue stent replacement to see if TPE and cholestyramine alone could control her pruritus. Thus far, she has not undergone stent replacement in 13 months and her pruritus remains under control. Furthermore, she has had no major adverse events with TPE, including no increase in infections. Serial hepatic function tests, performed since the initiation of TPE, show a progressive decrease in bilirubin and alkaline phosphatase levels with little to no effect on transaminases. In conclusion, this patient demonstrates the potential of TPE to alleviate symptoms and reduce invasive procedures and the use of multiple drugs in the management of intractable pruritus due to PSC. Thus, TPE might be a safe and effective alternative to treat this patient population.