Cholangitis refers to a chronic liver condition characterized by fibrosis, inflammation, and obliteration of the large bile duct. It is also referred to as primary sclerosing cholangitis (PSC).

The cause is unknown. Causes attributed to the condition include toxins such as copper; infectious agents; and abnormalities in immune regulation. The condition is common in young men, and it is also associated with inflammatory bowel disease such as ulcerative colitis.

The onset is usually not obvious. There is progressive fatigue, pruritus, and jaundice. In addition, there may be right upper quadrant pain and fever. The liver may be enlarged, or there may be characteristics of cirrhosis (see Diagnostic Code: 7312 Liver, cirrhosis of). Cirrhosis, portal hypertension, and liver failure characterize the terminal phase of the disease.

Diagnostic measures include blood tests focused on liver biochemistry and cholangiography. Liver biopsy may be used to confirm the diagnosis.

Treatment may include drug therapy, antibiotics, endoscopic or, transhepatic dilation for strictures. Liver transplantation is the most effective treatment.

Cirrhosis, portal hypertension, ascites, and liver failure are terminal outcomes of the illness. Patients with advanced conditions are prone to develop cholangiocarcinoma.

• None.

• Rate as for chronic cholecystitis.

• Ratings under diagnostic codes 7301 to 7329, inclusive, 7331, 7342, and 7345 to 7348 inclusive will not be combined with each other. A single evaluation will be assigned under the diagnostic code which reflects the predominant disability picture, with elevation to the next higher evaluation where the severity of the overall disability warrants such elevation.” [38 CR 4.114]

• There are diseases of the digestive system, particularly within the abdomen, which, while differing in the site of pathology, produce a common disability picture characterized in the main by varying degrees of abdominal distress or pain, anemia and disturbances in nutrition. Consequently, certain coexisting diseases in this area, as indicated in the instruction under the title “Diseases of the Digestive System,” do not lend themselves to distinct and separate disability evaluations without violating the fundamental principle relating to pyramiding as outlined in 38 CFR 4.14.  [38 CFR 4.113]