Primary sclerosing cholangitis - Types

Infectious sclerosing cholangitis

Even with the undoubted infectious nature of sclerosing cholangitis, according to biochemical, histological and cholangiographic signs, it may not differ in any way from primary sclerosing cholangitis.

Bacterial cholangitis

Bacterial cholangitis in the absence of mechanical, usually partial, biliary obstruction rarely develops. Most often, the infection is ascending, from the intestine. Obstruction of the bile ducts causes excessive growth of microflora in the upper parts of the small intestine.

Infiltration of polymorphonuclear leukocytes and destruction of the epithelium occur in the walls of the damaged ducts. Eventually, the bile duct is swept away by a fibrous cord. The cause may be gallstone disease, biliary strictures, and stenosis of the biliary-intestinal anastomosis. The loss of the bile ducts is irreversible. Once they are destroyed, biliary cirrhosis persists, even if the cause of the duct obstruction is eliminated (for example, by removing gallstones).

If an anastomosis is made between the common bile duct or hepatic duct and the duodenum, the constant flow of microorganisms from the intestine into the bile ducts can lead to bacterial cholangitis (blind sac syndrome) even in the absence of bile duct obstruction. Similar complications can develop after sphincteroplasty.

Infestation with Chinese liver fluke (Clonorchis sinensis), causing biliary obstruction, promotes the development of secondary infection, usually caused by Escherichia coli, which is the cause of sclerosing cholangitis.

Multiple purulent abscesses lead to the picture of sclerosing cholangitis.

Cholangitis caused by opportunistic microorganisms and immunodeficiency

Sclerosing cholangitis can develop when the bile ducts are affected by opportunistic microorganisms, usually against the background of congenital or acquired immunodeficiency.

In newborns, the biliary tract epithelium is selectively affected by CMV and reovirus type III, which cause obliterating cholangitis.

Immunodeficiency disorders that frequently cause cholangitis include familial combined immunodeficiency, immunodeficiency with hyperIgM production, angioimmunoblastic lymphadenopathy, X-linked immunodeficiency, and immunodeficiency with transient T-cell disorders. The disease is most often caused by CMV, cryptosporidia, or a combination of both. Infections with Cryptococcus, Candida albicans, and Klebsiella pneumoniae are also possible.

AIDS also causes biliary tract pathology. In a group of 26 AIDS patients with biliary tract pathology, 20 had significant changes on cholangiograms. Fourteen of them showed a picture of sclerosing cholangitis with or without stenosing papillitis.

Bile duct damage in primary sclerosing cholangitis and AIDS differs in the nature of inflammatory infiltration. In primary sclerosing cholangitis, the infiltrates contain a large number of T4 lymphocytes, while in AIDS this subpopulation of lymphocytes is absent.

Graft versus host disease

In rejection of the transplanted liver and graft-versus-host disease (GVHD) developing after allogeneic bone marrow transplantation, pathological expression of HLA class II antigens is detected in the bile ducts. Rejection is manifested by progressive non-purulent cholangitis, the most striking symptom of which is the disappearance of the interlobular bile ducts. The epithelium of the bile ducts is infiltrated with lymphocytes, foci of necrosis develop and its integrity is disrupted. Similar changes are detected in GVHD after allogeneic bone marrow transplantation. In one of these patients, severe cholestatic jaundice continued

10 years and during liver biopsy, progressive biliary fibrosis and cirrhosis were detected in dynamics. The patient died from liver failure.

Sclerosing cholangitis after liver transplantation

Sclerosing cholangitis may develop in a failed transplant. Liver biopsy is not always diagnostic, and changes may simply indicate obstruction of a large duct. Sclerosing cholangitis may be caused by transplant incompatibility, hepatic artery thrombosis, and chronic rejection.

Vascular cholangitis

The bile ducts are richly supplied with blood by the hepatic artery, which forms the peribiliary vascular plexus. Disruption of the blood supply leads to ischemic necrosis of the extra- and intrahepatic bile ducts and, ultimately, to their complete disappearance. Damage to large branches of the hepatic artery, for example during cholecystectomy, leads to ischemia of the duct wall, damage to their mucous membrane and the entry of bile into the wall, which causes fibrosis and stricture of the duct. Similar processes can occur during liver transplantation, especially if the recipient's duct segment is too short and, as a result, is deprived of arterial blood supply.

In rare cases, men with chronic transplant rejection develop bile duct ischemia due to thickening of the intima of the liver arterioles.

Disappearance of the bile ducts is also possible with systemic vasculitis with diffuse inflammation of small arteries.

For the treatment of metastases of rectal or colon cancer to the liver, floxuridine (5-FUDR) is administered into the hepatic artery using an infusion pump. It may be complicated by biliary strictures. The picture resembles primary sclerosing cholangitis. The process of disappearance of the bile ducts may be so pronounced that a liver transplant is necessary.

Drug-induced cholangitis

Cholangitis may occur when anthelmintic drugs are injected into an echinococcal cyst. Usually, the lesion is limited to only part of the bile ducts. Over several months, a stricture develops, leading to jaundice, biliary cirrhosis, and portal hypertension.

Histiocytosis X

A cholangiographic picture similar to that of primary sclerosing cholangitis can be observed in histiocytosis X. As the biliary tract lesion progresses, hyperplastic changes are replaced by granulomatosis, xanthomatosis, and finally fibrosis. The clinical picture is similar to that of primary sclerosing cholangitis.

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