The best approach for diagnosing primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome with autoimmune features and is associated with other immunological diseases such as autoimmune pancreatitis and inflammatory bowel disease. In addition to primary biliary cirrhosis, PSC is one of the most common chronic cholestatic liver diseases. The diagnosis of PSC is a challenge because patients are asymptomatic or display unspecific symptoms. Patients experience fatigue, pruritus, and jaundice. In addition, symptom onset is not associated with age. The diagnosis of PSC is typically achieved after a complication such as cholangitis or hepatic dysfunction occurs. Patients may experience periods of remission. Laboratory tests show cholestatic profiles with high levels of alkaline phosphatases (APs), gamma-glutamyltransferases (GGTs) and, occasionally, greater bilirubin levels. Definitive diagnosis is achieved using imaging methods to assist in the differential diagnosis. Endoscopic retrograde cholangiography (ERC) has been considered the gold-standard diagnostic method for PSC. However, ERC has its inherent risks such as acute pancreatitis, cholangitis, and duodenum perforation. Moreover, in a patient with biliary chronic disease, the loss of the Oddi sphincter barrier may lead to acute complications and chronic problems such as recurrent cholangitis, liver abscesses, and reduced liver function. Magnetic resonance imaging (MRI) is an attractive option for PSC diagnosis. Many studies have reported that MRI has similar sensitivity to ERC for PSC and has the remarkable advantages of being less invasive and less likely to cause complications. In this case report, we present two patients with PSC who had serious complications after ERC with recurrent cholangitis and are on the waiting list for a liver transplant. CASE 1: A 51-year-old male patient with a previous diagnosis of inflammatory bowel disease (IBD) and complaints of chronic fatigue and pruritus was referred to our hospital. Liver function tests showed normal transaminase levels and a two-fold increase in the alkaline phosphatase and gamma-glutamyl transpeptidase levels. A cholangio-NMR (c-NMR) study showed a pattern of stenosis and dilation that suggested a diagnosis of primary sclerosing cholangitis (PSC) (Figure 1). The patient underwent ERCP with papillotomy, which confirmed this diagnosis. After three days, the patient presented with a fever, leukocytosis, and chills. He developed multiple intra-hepatic abscesses. Acute cholangitis was diagnosed and successfully treated with long-term IV antibiotics. After three years, the patient experienced three more episodes of cholangitis, which were treated with IV antibiotics. He is currently on daily prophylactic treatment with ciprofloxacin and remains asymptomatic. CASE 2: A 39-year-old woman started having diarrhea, hepatomegaly, jaundice, and elevated canalicular enzymes seven years ago. An ERCP with papillotomy was performed, and the findings strongly supported the diagnosis of PSC (Figure 2). Since then, the patient has experienced at least four major attacks of acute cholangitis that necessitated inpatient treatment. She is currently receiving prophylactic ciprofloxacin daily and is asymptomatic. However, she developed hepatic cirrhosis with portal hypertension. Three months ago, she experienced upper gastrointestinal bleeding due to esophageal varices. Liver transplantation has been indicated, and her current MELD score is 17.