Secondary sclerosing cholangitis (SSC) is a chronic cholestatic liver disease. SSC is a sclerosing cholangitis with a known cause. Alternatively, if no cause can be identified, then

primary sclerosing cholangitis Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder. Affected individuals may ...

is diagnosed. SSC 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.

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 Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery ...

blunt Blunt may refer to: * Blunt (surname), a surname (and list of people with the name) * Blunt (cigar), a term used in the cigar industry to designate blunt-tipped, usually factory-rolled cigars * Blunt (cannabis), a slang term used in cannabis cult ...

abdominal trauma Abdominal trauma is an injury to the abdomen. Signs and symptoms include abdominal pain, tenderness (medicine), tenderness, rigidity, and bruise, bruising of the external abdomen. Complications may include blood loss and infection. Diagnosis ma ...

, intra-

arterial An artery () is a blood vessel in humans and most other animals that takes oxygenated blood away from the heart in the systemic circulation to one or more parts of the body. Exceptions that carry deoxygenated blood are the pulmonary arteries in ...

chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

, and recurrent

pancreatitis Pancreatitis is a condition characterized by inflammation of the pancreas. The pancreas is a large organ behind the stomach that produces digestive enzymes and a number of hormone A hormone (from the Ancient Greek, Greek participle , "se ...

. It has been clearly demonstrated that sclerosing cholangitis can develop after an episode of severe bacterial cholangitis. Also it was suggested that it can result from insult to the biliary tree by obstructive cholangitis secondary to

choledocholithiasis Common bile duct stone, also known as choledocholithiasis, is the presence of gallstones in the common bile duct (CBD) (thus '' choledocho-'' + '' lithiasis''). This condition can cause jaundice and liver cell damage. Treatments include choledocho ...

, surgical damage, trauma, vascular insults,

parasites Parasitism is a close relationship between species, where one organism, the parasite, lives (at least some of the time) on or inside another organism, the host, causing it some harm, and is adapted structurally to this way of life. The en ...

, or congenital fibrocystic disorders. Additional causes of secondary SC are toxic, due to chemical agents or drugs.

Diagnosis

SSC is clinically related to

( PSC), but originates from a known pathological process. Diagnosis of PSC requires the exclusion of all secondary causes of sclerosing cholangitis; else, if a known aetiology can be uncovered, SSC is diagnosed. 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. Serologic testing, radiological imaging and histological analysis can help diagnose SSC.

Treatment

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

References

External links

{{Digestive system diseases Biliary tract disorders Hepatology Inflammations