Pruritus in Primary Sclerosing Cholangitis: New Insights into Cause and Treatment
Pruritus is a symptom commonly experienced by patients with primary sclerosing cholangitis (PSC), ranging in severity from mild and unobtrusive to being profoundly debilitating. However, the etiology of pruritus in PSC patients remains poorly understood,
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Mark G. Swain
Introduction Pruritus is a common complaint among patients with cholestatic liver diseases. Specifically, pruritus is a distinct and profound symptom associated with intrahepatic cholestasis of pregnancy (ICP) and benign recurrent intrahepatic cholestasis (BRIC). Moreover, pruritus is commonly encountered in patients with primary biliary cholangitis (PBC), affecting up to three-fourths of PBC patients to some degree [1]. In patients with PBC, itch can also be severe, significantly impairing patient quality of life (QOL) leading to depression, social withdrawal, and even suicidal ideation. In rare cases, severe itch can even be an indication for liver transplantation [1, 2]. In contrast, the prevalence and impact of pruritus in PSC patients are less well understood. The prevalence of pruritus in PSC patients at the time of diagnosis has been reported for a number of wellcharacterized patient cohorts. In Scandinavia, in a cohort of 305 patients with PSC, 30 % had pruritus at the time of their diagnosis [3]. However, in a cohort of PSC patients followed at the Mayo Clinic in Rochester, Minnesota [4], pruritus was almost twice as common at the time of diagnosis (59 %)
M.G. Swain, MD, MSc, FRCPC, FAASLD Division of Gastroenterology and Hepatology, Department of Medicine, University of Calgary, Calgary, AB, Canada e-mail: [email protected]
compared to the frequency reported by Broome et al. [3]. This discrepancy likely reflects the specialized referral pattern for PSC patients seen at the Mayo Clinic. Moreover, among the Mayo patient cohort, 75 % of the patients who were symptomatic at diagnosis reported pruritus [4]. In another Scandinavian study, 65 PSC patients were provided with daily diaries and asked to report symptoms over a 3-year period [5]. A majority of patients (84 %) reported the occurrence of symptoms during this period, including pruritus, however these symptoms were typically intermittent and transient (lasting 1–2 days). In these patients, pruritus correlated closely with serum alkaline phosphatase levels [5]. Berquist et al. [6] examined a cohort of 246 PSC patients and divided them into those diagnosed before (n = 185) and after (N = 61) 1998. At the time of PSC diagnosis, 20 % of patients complained of pruritus. Interestingly, pruritus in these patients was significantly more common in women (28 %) than in men (16 %), a finding paralleling observations from PBC patients where women are more likely to be pruritic than men [7]. These observations are suggestive of hormonal regulation of pruritus in cholestasis and are consistent with the common clinical observation that pruritus in PBC patients often worsens around the time of menses. Perhaps not surprisingly, pruritus was reported in 25 % of patients diagnosed with PSC by endoscopic retrograde cholangiopancreatography (ERCP), compared to 5 % of patients diagnosed using magnetic resonance cholangiopancreatography (MRCP). The frequency of pruritus at the time of diagnosis
© Springer International Publishing Switzerland 2017 L.M. For
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