Primary Sclerosing Cholangitis (PSC) is a rare chronic liver disease characterized by inflammation and scarring of the bile ducts inside and outside the liver. It primarily affects middle-aged individuals, with a slight male predominance. PSC is more prevalent in Northern Europe and North America, particularly among individuals of Northern European descent.
Estimating the exact prevalence of PSC is challenging due to its rarity and varying diagnostic criteria. However, studies suggest that the prevalence ranges from 6 to 16 cases per 100,000 individuals in the general population. The incidence of PSC appears to be increasing over time, possibly due to improved diagnostic techniques and awareness.
PSC is strongly associated with other autoimmune conditions, such as inflammatory bowel disease (IBD), particularly ulcerative colitis. Approximately 70-80% of PSC patients also have IBD. The co-occurrence of PSC and IBD can worsen the prognosis and increase the risk of complications.
While PSC can occur at any age, it most commonly presents between the ages of 30 and 50. The disease progresses slowly, and patients may remain asymptomatic for years. However, over time, PSC can lead to liver cirrhosis, liver failure, and an increased risk of developing bile duct and liver cancers.
Early detection and management of PSC are crucial to improve patient outcomes. Regular monitoring, symptom management, and potential liver transplantation are key aspects of PSC treatment.