From the Guidelines
Primary sclerosing cholangitis (PSC) often presents with no symptoms in its early stages, but as the disease progresses, patients typically develop fatigue, itching (pruritus), jaundice (yellowing of the skin and eyes), abdominal pain in the upper right quadrant, unexplained weight loss, and episodes of fever with chills, as reported in the most recent and highest quality study 1. The symptoms of PSC can be variable, but common symptoms include:
- Right upper quadrant abdominal discomfort
- Fatigue
- Pruritus
- Weight loss
- Episodes of cholangitis, characterized by fever and chills, although this is uncommon at presentation 1
- Jaundice
- Dark urine
- Pale-colored stools
- Enlarged liver or spleen
Advanced PSC can lead to complications, including:
- Bacterial cholangitis (bile duct infections) characterized by severe fever, pain, and jaundice
- Portal hypertension causing varices and ascites
- Metabolic bone disease resulting in osteoporosis Approximately 70-80% of PSC patients also have inflammatory bowel disease, particularly ulcerative colitis, which may cause additional symptoms like diarrhea and rectal bleeding 1. The diagnosis of PSC is based on the combination of clinical, laboratory, imaging, and histological findings, with magnetic resonance cholangiopancreaticography (MRCP) being the preferred diagnostic test 1. PSC symptoms develop because the disease causes progressive inflammation, scarring, and narrowing of the bile ducts, both inside and outside the liver, which impairs bile flow and leads to bile accumulation in the liver, potentially progressing to cirrhosis and liver failure in advanced cases 1.
From the Research
Symptoms of Primary Sclerosing Cholangitis
The symptoms of primary sclerosing cholangitis (PSC) can vary, but common presenting symptoms include:
Disease Characteristics
PSC is a chronic, cholestatic liver disease characterized by inflammatory destruction of the intrahepatic and/or extrahepatic bile ducts, leading to bile stasis, fibrosis, and ultimately to cirrhosis 2, 4. It is often associated with inflammatory bowel disease (IBD) and can increase the risk of various malignancies, particularly cholangiocarcinoma 2, 4, 5.
Diagnosis and Treatment
Diagnosis of PSC is usually established by cholangiography, and serum biochemical tests indicate cholestasis 3. While there is no curative treatment for PSC, ursodeoxycholic acid (UDCA) may be used to improve biochemical parameters of cholestasis, although its effectiveness is still debated 4, 6. Liver transplantation is currently the only life-extending therapeutic approach for eligible patients with end-stage PSC 2, 4, 3, 5.