Secondary sclerosing cholangitis

Secondary sclerosing cholangitis (SSC) is a chronic cholestatic liver disease. It is an aggressive and rare disease with complex and multiple causes. It is characterized by inflammation, fibrosis, destruction of the biliary tree and biliary cirrhosis. It can be treated with minor interventions such as continued antibiotic use and monitoring, or in more serious cases, laparoscopic surgery intervention, and possibly a liver transplant.

Secondary sclerosing cholangitis
SpecialtyInternal medicine

Cause

SSC is thought to develop as a consequence of known injuries or pathological processes of the biliary tree, such as biliary obstruction, surgical trauma to the bile duct, or ischemic injury to the biliary tree. Secondary causes of SSC include intraductal stone disease, surgical or blunt abdominal trauma, intra-arterial chemotherapy, and recurrent pancreatitis.[1] It has been clearly demonstrated that sclerosing cholangitis can develop after an episode of severe bacterial cholangitis.[2] Also it was suggested that it can result from insult to the biliary tree by obstructive cholangitis secondary to choledocholithiasis, surgical damage, trauma, vascular insults, parasites, or congenital fibrocystic disorders. Additional causes of secondary SC are toxic, due to chemical agents or drugs.[3][4]

Diagnosis

The diagnosis of SSC requires the exclusion of secondary causes of sclerosing cholangitis and recognition of associated conditions that may potentially imitate its classic cholangiographic features. It is morphologically similar to primary sclerosing cholangitis (PSC) but originates from a known pathological process. Its clinical and cholangiographic features may mimic PSC, yet its natural history may be more favorable if recognition is prompt and appropriate therapy is introduced. Sclerosing cholangitis in critically ill patients, however, is associated with rapid disease progression and poor outcome.[5] Serologic testing, radiological imaging and histological analysis can help diagnose SSC.[6]

Treatment

First lines of treatment can include mass spectrum antibiotics or drainage of the bile duct that is infected followed by close monitoring.[7] Endoscopic surgery is favored over open procedures to reduce infection and quicker recovery times. If these fail a liver transplant may be necessary.

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References
  1. Abdalian R, Heathcote EJ (November 2006). "Sclerosing cholangitis: a focus on secondary causes". Hepatology. 44 (5): 1063–74. doi:10.1002/hep.21405. PMID 17058222.
  2. ter Borg PC, van Buuren HR, Depla AC (June 2002). "Bacterial cholangitis causing secondary sclerosing cholangitis: a case report". BMC Gastroenterology. 2: 14. doi:10.1186/1471-230X-2-14. PMC 116430. PMID 12057011.
  3. Kariv R, Konikoff FM (December 2002). "Sclerosing cholangitis--primary, secondary and more.". The Israel Medical Association Journal : IMAJ. 4 (12): 1141–2. PMID 12516911.
  4. "Secondary sclerosing cholangitis". Retrieved 6 January 2017.
  5. "Causes of Secondary Sclerosing Cholangitis". Retrieved 6 January 2017.
  6. "Secondary Sclerosing Cholangitis due to Gallbladder Adenocarcinoma". Retrieved 6 January 2017.
  7. Björnsson, Einar S.; Gudnason, Hafsteinn O. (2017-06-23). "Secondary sclerosing cholangitis in critically ill patients: current perspectives". Clinical and Experimental Gastroenterology. Retrieved 2019-06-21.
Classification
  • ICD-10: K83.0
External resources
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