Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by progressive inflammation and fibrosis of the biliary tract, evolving to cirrhosis. It is commonly associated with inflammatory bowel disease (IBD). Aim: To communicate the clinical characteristics of patient with PSC seen in two reference centers. Patients and methods: Review of medical records of patients with PSC confirmed by liver biopsies. The clinical picture, laboratory abnormalities, imaging studies and treatment were recorded. Results: Thirty three patients (aged 16 to 73 years, 64% female) were identified. They corresponded to 1.7% of liver biopsies done between 1991-2003. Clinical presentation was a cholestatic picture in 40%, right upper abdominal pain in 36%, a dysenteric syndrome in 9% and varied symptoms in 15%. Laboratory tests showed cholestasis in 94% and positive anti ANCA, SMA, ANA and AMA antibodies in 28, 18, 15 and 9% of cases, respectively. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography were diagnostic in 43 and 58% of patients, respectively. There was an association with ulcerative colitis in 12% of cases. Liver biopsies showed grade I PSC in 76% and grade II-III in 6% of patients. It also showed a concomitant chronic hepatitis and primary biliary cirrhosis in 12 and 6% of cases, respectively. Treatment consisted on ursodeoxycholic acid (UDCA) in 45%, UDCA plus 5-aminosalicylic acid derivatives in 12% and UDCA plus immunosuppresors in 12% of patients. Two patients had to be transplanted. Conclusions: PSC is an uncommon cause of chronic liver disease. It is suspected in cholestatic patients and confirmed with a liver biopsy. It can be associated with other autoimmune hepatic and extrahepatic diseases.
Orellana N, I., Valera M, J. M., Nei L, C., Poniachik T, J., Berger F, Z., Latorre M, R., … Brahm B, J. (2005). Colangitis esclerosante primaria: Revisión de 12 años en dos centros de referencia. Revista Medica de Chile. https://doi.org/10.4067/s0034-98872005000700004