Abstract
The primary sclerosing cholangitis (PSC) is a progressive, idiopathic, immune mediated disease, characterized by intra or extrahepatic cholestasis, Inflammation and fibrosis of the bile ducts, which may progress to liver cirrhosis or cholangiocarcinoma (CCA) in the vast majority of patients. Objective: Analyze what the current literature discusses about the management of PSC. Method: A systematic review was carried out using the PubMed and SciELO database, applying the descriptors "Primary Sclerosing Cholangitis" AND "Cholangite" AND "Clinical management" AND Cholangiocarcmoma. Discussion: It is more common in men, with an average diagnosis at 35 years old. The PSC has a wide range of symptoms and stages of the disease. In the beginning, symptoms are rare and patients may be asymptomatic. The most commonly found symptoms are pruritus, fatigue, right upper quadrant abdominal pain, jaundice, fever, and weight loss. In about 75% of patients with PSC, liver function tests are chronically abnormal and fluctuating with a most commonly found pattern of cholestatic condition with elevation of alkaline phosphatase (AF) and yglutamyl transferase (GGT). Although endoscopic retrograde cholangiopancreatography (ERCP) is conventionally considered the gold standard test for the diagnosis of PSC, the main modality of choice currently for Imaging diagnosis in patients with suspected PSC is magnetic resonance cholangiopancreatography (CPRM). Due to the absence of effective medical therapy for PSC, liver transplantation (LT) remains the only curative option. There is little evidence for the use of medications as a way to inhibit the progression of the disease. Still several new drugs are under investigation, representing the potential to improve survival and clinical outcomes in PSC. Final considerations: We conclude that it is extremely important to approach the management of PSC. The diagnosis is a great challenge since its clinical, laboratory and imaging aspects overlap, as it was portrayed during this exposure. Nevertheless, due to the various differential diagnoses ofjaundice with cholestatic pattern, the etiological search is crucial and the absence of effective medical therapy, with several new drugs under analysis, should guide further studies to improve the survival and clinical outcomes of these patients. Another point to be considered is that there is a scarcity of data in the literature that address the PSC in the Brazilian population.
Published Version
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