Primary sclerosing cholangitis
Primary sclerosing cholangitis is an autoimmune fibroinflammatory disease of the large bile ducts, occurring mostly in young men and associated with inflammatory bowel disease, chiefly ulcerative colitis, in 80 to 90% of cases. MRCP showing multifocal strictures and dilations confirms the diagnosis, no drug alters progression, and management centers on endoscopic dilation of dominant strictures, surveillance for cholangiocarcinoma and colorectal cancer, and liver transplantation for advanced disease.
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What this article covers
- Overview
Definition: primary sclerosing cholangitis (PSC) is a chronic autoimmune fibroinflammatory disease of the large intrahepatic and extrahepatic bile ducts that produces multifocal strictures, upstream…
- Epidemiology
Male predominance: men account for about 70% of cases, unique among the autoimmune liver diseases, with median age at diagnosis around 40 years.
- Risk factors
Ulcerative colitis: the single strongest association; suspect PSC in any patient with IBD who develops an elevated alkaline phosphatase.
- Pathophysiology
Immune-mediated bile duct injury: an immune mechanism drives progressive inflammation and fibrosis of the bile duct wall, but the exact trigger remains unknown.
- Clinical features
Often asymptomatic at onset: many patients are identified by an incidental elevation of alkaline phosphatase and gamma-glutamyl transferase, particularly during evaluation of known IBD.
- Differential diagnosis
Primary biliary cholangitis: affects small and medium intrahepatic ducts, 9-to-1 female predominance, antimitochondrial antibody (AMA) positive, normal cholangiogram (primary biliary cholangitis).
- Diagnosis
Confirm with cholangiography, not a formal scoring system: PSC is established by MRCP showing multifocal strictures and segmental dilation of the intrahepatic and/or extrahepatic bile ducts, the…
- Management
No drug halts disease progression: PSC has no proven disease-modifying therapy; ursodeoxycholic acid's use to slow progression is controversial and unproven to improve transplant-free survival…
- Complications
Cholangiocarcinoma: the most feared complication, 15% lifetime risk, incidence highest in the first 2 years after diagnosis, and often hard to distinguish from a benign dominant stricture.
- Prognosis
Median transplant-free survival is about 12 years from diagnosis, among the shortest of the autoimmune liver diseases, highlighting the eventual need for transplantation in most patients.
- Prevention
Colonoscopy at PSC diagnosis, then annually: all patients with PSC and coexisting IBD (or those found to have occult IBD at diagnostic colonoscopy) require surveillance colonoscopy with biopsies…
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Related articles
- Primary biliary cholangitis
Primary biliary cholangitis is an autoimmune, T cell-mediated destruction of small and medium intrahepatic bile ducts that presents with fatigue and pruritus in middle-aged women and a cholestatic liver chemistry pattern. Diagnosis requires two of three criteria (elevated alkaline phosphatase, positive antimitochondrial antibody, compatible biopsy), and lifelong ursodeoxycholic acid is first-line treatment, with obeticholic acid or fibrates added for inadequate biochemical response.
- Ulcerative colitis
Ulcerative colitis is a chronic immune-mediated disease of continuous mucosal inflammation that begins in the rectum and extends proximally through the colon, presenting with bloody diarrhea and tenesmus and confirmed by colonoscopy with biopsy. Treatment is stepped by extent and severity from 5-aminosalicylates through corticosteroids, immunomodulators, and biologics, with acute severe disease requiring IV corticosteroids and medical or surgical rescue, and colectomy remains curative.
- Colorectal cancer
Colorectal cancer is an adenocarcinoma that arises through the adenoma-carcinoma sequence and presents differently by location, with right-sided tumors causing occult bleeding and iron-deficiency anemia and left-sided tumors causing obstruction and hematochezia. Colonoscopy with biopsy confirms the diagnosis, TNM staging on CT chest/abdomen/pelvis with CEA guides treatment, and management ranges from surgical resection alone for localized disease to adjuvant FOLFOX for node-positive colon cancer, neoadjuvant chemoradiation for rectal cancer, and molecularly targeted systemic therapy for metastatic disease.
- Acute cholangitis
Acute cholangitis is a bacterial infection of an obstructed biliary tree, classically presenting as Charcot triad of fever, right upper quadrant pain, and jaundice and progressing to septic shock when drainage is delayed. Diagnosis follows the Tokyo Guidelines and severity grade, and treatment combines prompt broad-spectrum antibiotics with biliary drainage, most often by ERCP.
- Cirrhosis
Cirrhosis is end-stage hepatic fibrosis with regenerative nodules, staged as compensated or decompensated by the onset of ascites, variceal hemorrhage, encephalopathy, or jaundice. Severity is graded by Child-Pugh class and MELD, and management centers on treating the underlying cause, surveillance for varices and hepatocellular carcinoma, and timely transplant referral once decompensation appears.
- Portal hypertension
Portal hypertension is elevated pressure in the portal venous system, most often from cirrhosis, that drives the major decompensating complications of chronic liver disease: varices, ascites, splenomegaly, and encephalopathy. Diagnosis rests on imaging and endoscopy for its downstream signs, and management combines treating the underlying cause with nonselective beta-blockade and, for refractory complications, TIPS.