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Table 2 Primary sclerosing cholangitis

From: Patient and clinician opinions of patient reported outcome measures (PROMs) in the management of patients with rare diseases: a qualitative study

Primary sclerosing cholangitis (PSC) is a progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts [17]. This may lead to the accumulation of bile which in turn may lead to liver damage and in the long term, cirrhosis, portal hypertension, liver tumours and liver failure [17].
While PSC may occur at any age, it is more frequent in adults 30–60 years old. PSC is approximately twice as common in men [18]. It is estimated that 70–80% of patients with PSC have inflammatory bowel diseases such as ulcerative colitis and Crohn’s disease [19].
PSC is associated with a high level of morbidity which may have a significant impact on HRQOL [20]. Tiredness or fatigue may be an early symptom while pruritus, jaundice, abdominal pain, weight loss, fevers, hyperpigmentation, vitamin deficiencies and metabolic bone disease may occur as disease progresses [21].
The aim of medical treatment is to control symptoms and manage any complications. Currently, the only clinically proven treatment for severely damaged liver is transplantation [17]. However, after transplantation, there is a likelihood of recurrence and acute rejection [22].