Primary sclerosing cholangitis: types

Infectious sclerosing cholangitis

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

Bacterial cholangitis

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

Inflammation of polymorphonuclear leukocytes and destruction of the epithelium occur in the walls of the damaged ducts. In the end, the bile duct swells with a fibrous cuff. The cause may be cholelithiasis, strictures of the biliary tract and stenosis of the biliary-intestinal anastomosis. The loss of the bile ducts is irreversible. After their destruction, biliary cirrhosis persists, even if the cause of obstruction of ducts is eliminated (for example, stones of the bile ducts are removed).

If an anastomosis is placed between the common bile duct or the liver duct and the duodenum, the constant flow of microorganisms from the intestine into the bile ducts may lead to bacterial cholangitis (the "blind bag" syndrome) in the absence of obstruction of the bile ducts. Similar complications may develop after sphincteroplasty.

The invasion of the Chinese hepatic ligament (Clonorchis sinensis), causing biliary obstruction, contributes to the development of a secondary infection, usually caused by Escherichia coli, which is the cause of sclerosing cholangitis.

Multiple purulent abscesses lead to a picture of sclerosing cholangitis.

Cholangitis caused by opportunistic microorganisms and caused by immunodeficiency

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

In neonates, the epithelium of the biliary tract is selectively affected by CMV and type III rheovirus, which cause obliterating cholangitis.

Immunodeficiency disorders, in which cholangitis is often observed, include familial combined immunodeficiency, immunodeficiency with hyperproduction of IgM, angioimmunoblastic lymphadenopathy, immunodeficiency linked to the X chromosome, and immunodeficiency with transient T-cell disorders. More often the disease is caused by CMV, cryptosporidia, or a combination of them. Infection with Cryptococcus, Candida albicans and Klebsiella pneumoniae is possible .

With AIDS, the pathology of the biliary tract also develops. In a group of 26 patients with AIDS with pathology of the biliary tract in 20, cholangiograms showed significant changes. 14 of them had a picture of sclerosing cholangitis with or without stenosing papillitis.

The defeat of the bile ducts with primary sclerosing cholangitis and AIDS differs in the nature of the inflammatory infiltration. When primary sclerosing cholangitis in infiltrates contains a large number of T4-lymphocytes, with AIDS, this particular subpopulation of lymphocytes is absent.

Graft versus host disease

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

10 years and with biopsy of the liver, progressive biliary fibrosis and cirrhosis were found in the dynamics. The patient died from liver failure.

Sclerosing cholangitis after liver transplantation

Sclerosing cholangitis can develop in an inconsistent transplant. With liver biopsy, the diagnosis can not always be made, changes may simply indicate obstruction of a large duct. The causes of sclerosing cholangitis may be incompatibility of the transplant, thrombosis of the hepatic artery and chronic rejection.

Vascular cholangitis

The bile ducts are abundantly blood flowing with the hepatic artery, which forms the peribiliary vascular plexus. Disturbance of blood supply leads to ischemic necrosis of extra- and intrahepatic bile ducts and eventually to their complete disappearance. Damage of large branches of the hepatic artery, for example, with cholecystectomy, leads to ischemia of the duct wall, damage to their mucous membrane and bile in the wall, which causes fibrosis and stricture of the duct. Similar processes may occur in liver transplantation, especially if the segment of the recipient's duct is too short and, as a result, is deprived of an arterial blood supply.

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

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

For the treatment of metastasis of cancer of the rectum or colon, the liver is administered to the liver artery by means of a flocsuridine infusion pump (5-FUDR). It can be complicated by strictures of the bile ducts. The picture resembles the primary sclerosing cholangitis. The process of disappearance of the bile ducts can be so pronounced that there is a need for liver transplantation.

Medicinal cholangitis

Cholangitis may occur with the administration of anthelmintic drugs in the echinococcal cyst. Usually, the defeat is limited to only a part of the biliary tract. Within a few months, stricture develops, leading to jaundice, biliary cirrhosis and portal hypertension.

Histiocytosis X

A cholangiographic pattern similar to that of primary sclerosing cholangitis can be observed with histiocytosis X. With progression of the lesions of the biliary tract, hyperplastic changes are followed by granulomatosis, xanthomatosis and, finally, fibrosis. The clinical picture is similar to the picture of primary sclerosing cholangitis.

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