Vanishing bile duct syndrome vbds refers to a group of acquired disorders associated with progressive destruction and disappearance of the intrahepatic bile ducts leading to cholestasis. The mechanisms of this severe complication have been poorly understood until now. A rare case of ibuprofenassociated vanishing bile duct syndrome in an adult female patient is presented. The possibility of a vanishing bile duct syndrome should be considered in the differential diagnosis of unexplained intrahepatic cholestasis in patients with hodgkins disease. Bile is a very alkaline liquid made in liver, it is stored in the gallbladder and secreted by common bile duct into. Vanishing bile duct syndrome associated with chronic ebv. Vbds typically occurs after a bout of severe cholestatic hepatitis, often with immunoallergic features. Vanishing bile duct syndrome request pdf researchgate.
Vanishing bile duct syndrome vbds is a rare disorder that leads to progressive loss and destruction of intrahepatic bile ducts and severe ductopenia or bile duct paucity. Vanishing bile duct syndrome with hyperlipidemia after. Antibiotic is one of the most frequently reported causes of vbds. Chemotherapy, hodgkins lymphoma, vanishing bile duct syndrome received. Vanishing bile duct syndrome with eruptive xanthomas cotter. Summary we reported here an adult patient with vanishing bile duct syndrome due to chronic ebv infection. Loss of bile ducts is a characteristic feature of chronic rejection in the liver allograft also known as irreversible rejection and vanishing bile duct syndrome. Vanishing bile duct syndrome arising in a patient with t. Hepatic ductopenia and vanishing bile duct syndrome uptodate. Although the pathogenesis is poorly understood, vbds has been associated with potential infectious etiologies, ischemia, autoimmune diseases, adverse drug reactions, and humoral factors. The syndrome consists primarily of severe icterus and hepatic laboratory abnormalities as a consequence of liver parenchyma that has undergone loss of normal bile ducts, hence the name of the syndrome. A case report of vanishing bile duct syndrome after. Pdf vanishing bile duct syndrome in hodgkins disease. A 63yearold woman was referred to our department with progressive cholestasis and hyperbilirubinaemia following a course of flucloxacillin.
Vanishing bile duct syndrome in a child with toxic. Vanishing bile duct syndrome vbds represents the final, irreversible stage of a variety of ductopenic diseases due to progressive intrahepatic bile duct loss. Despite the scarcity of cases, vbds is a recognised complication of cholestatic druginduced liver injury dili. Many factors are associated with vbds including infections, neoplasms, and drugs. Pdf vanishing bile duct syndrome vbds has been described in different pathologic conditions including infection, ischemia, adverse drug reactions. Metabolic medicine and chemical pathology, kings college hospital, london, uk. Vanishing bile duct syndrome related to dili and hodgkin. Pdf valproic acidassociated vanishing bile duct syndrome. Vanishing bile duct syndrome and inflammatory pseudotumor associated with a case of anabolic steroid. Vanishing bile duct syndrome vbds is a group of diseases that is characterized by the missing of bile duct in portal area. Vanishing bile duct syndrome secondary to antiretroviral. Vanishing bile duct syndrome is an unusual entity characterized by progressive destruction of the intrahepatic bile ducts ductopenia, which ultimately results in cholestasis.
Vanishing bile duct syndrome vbds is a unique clinicalpathologic entity with numerous etiologies. Ursodeoxycholic acid treatment of vanishing bile duct. Important advances have recently been made in the biology, physiology and treatment of vanishing bile duct syndrome. Lab hyperlipidaemia, fatsoluble vitamin a, d, e, k. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. Acute vanishing bile duct syndrome after therapy with cephal. Stevensjohnson syndrome is a hypersensitivity disorder that may also be caused by medications.
Vanishing bile duct syndrome refers to a group of acquired disorders resulting in progressive destruction and disappearance of the intrahepatic. These observations suggest that hodgkins lymphoma should be included in the list of diseases associated with loss of intrahepatic bile ducts. Despite the comprehensive laboratory, imaging and genetic investigations, no other cause for the. Vanishing bile duct syndrome vbds is characterized by cholestasis and progressive destruction of the intrahepatic bile ducts ductopenia. Hodgkin lymphomarelated vanishing bile duct syndrome and. Pdf vanishing bile duct syndrome in hodgkins lymphoma. The results found extensive damage of interlobular bile. Vanishing bile duct syndrome refers to a group of acquired disorders resulting in progressive destruction and disappearance of the intrahepatic bile ducts and ultimately cholestasis. Vanishing bile duct syndrome vbds defines a clinicopathological complex in which cholestasis is associated with loss of the intrahepatic bile ducts or ductopenia. Vanishing bile duct syndrome in a hodgkins lymphoma patient. Acute vanishing bile duct syndrome after therapy with. To describe a rare case of intrahepatic cholestasis due to vanishing bile duct syndrome.
The mechanism by which biliary epithelial cells are damaged and intrahepatic bile ducts are lost has not been fully elucidated. The characteristic histological features of this syndrome may also be seen in primary biliary cirrhosis, primary sclerosing cholangitis, graftvs. Vanishing bile duct syndrome an overview sciencedirect. Successful liver transplantation for druginduced vanishing. Ursodeoxycholic acid treatment of vanishing bile duct syndromes. Vanishing bile duct syndrome livertox ncbi bookshelf. Vanishing bile duct syndrome in a hodgkins lymphoma patient with fatal outcome despite lymphoma remission. Vanishing bile duct syndrome secondary to antiretroviral therapy in hiv 30 july 2010 vanishing bile duct syndrome vbds is an important cause of jaundice, and results from. Ductopenia a pathologic description refers to the associated reduction in the number of intrahepatic bile ducts, a process that ultimately leads to cholestasis. Regardless of the underlying etiology, vbds will ultimately lead to chronic cholestasis, biliary fibrosis, and ultimately cirrhosis or liver failure.
Vanishing bile duct syndrome in a hodgkins lymphoma. Amoxicillinclavulanic acid associated intrahepatic cholestasis responsive to ursodeoxycholic acid. Jun 14, 2019 here, we report a case of severe druginduced liver injury requiring liver transplantation due to vanishing bile duct syndrome vbds after exposure to pexidartinib in the ispy 2 trial, a phase 2. Vanishing bile duct syndrome vbds has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. Intrahepatic cholestasis in a possible mechanism hodgkins lymphoma for stefan g. Druginduced cholestasis has a wide range of clinical presentations, and in a small number of patients, it can progress to severe ductopenia. Vanishing bile duct syndrome is a severe cholestatic disease associated with toxic effects of medications. Vanishing bile duct syndrome is a loose collection of diseases which leads to the injury to hepatic bile ducts and eventual ductopenia. Vanishing bile duct syndrome vbds consists of a series of diseases characterized by the loss of 50% bile duct in portal areas. It is characterized by chronic abdominal pain, fever and cholestasis with dilation and irregularities of the bile ducts, both intra and extrahepatic fig. Vanishing bile duct syndrome vbds is a rare but serious outcome and complication of drug induced liver injury marked clinically by chronic cholestasis and histologically by loss of intrahepatic bile ducts. Clinicians need to be aware of vanishing bile duct syndrome as a serious consequence of ibuprofen use in adult patients, although ibuprofen is considered to be among the safest nsaids.
Here, we report a case of severe druginduced liver injury requiring liver transplantation due to vanishing bile duct syndrome vbds after exposure to pexidartinib in the i. Chronic cholestasis is the main feature of vanishing bile duct syndromes vbds characterized by progressive loss of small intrahepatic ducts caused by a variety of different diseases. Kai rou tey, md,a kristen barrett, md,b richa jain. Vanishing bile duct syndrome following treatment of ocular toxoplasmosis with pyrimethamine and tmpsmx. We report a 7monthold infant with ibuprofen associated toxic epidermal necrolysis, followed by severe and rapidly progressive vanishing bile duct syndrome. Vanishing bile duct syndrome is a rare acquired condition, characterized by progressive loss of intrahepatic bile ducts leading to ductopenia and cholestasis. Vanishing bile duct syndrome following treatment of ocular. Despite the comprehensive laboratory, imaging and genetic investigations, no other cause for the cholestasis was demonstrated. With particular reference to its relationship to primary biliary cirrhosis. Ibuprofen associated acute vanishing bile duct syndrome. Request pdf vanishing bile duct syndrome vanishing bile duct syndrome.
Vanishing bile duct syndrome vbds has been reported in a number of conditions such as drug administration, toxins, primary biliary cirrhosis, chronic allograft rejection, graftversushost disease, langerhans cell histiocytosis and alagille syndrome. Vanishing bile duct syndrome vbds consists of a series of diseases characterized by t. Vanishing bile duct syndrome in a child with toxic epidermal. It can be associated with infections, ischemia, drug adverse reactions, neoplasms, autoimmune disease, and allograft rejection.
Vanishing bile duct syndrome and inflammatory pseudotumor associated with a case of anabolic steroid abuse. Valproic acidassociated vanishing bile duct syndrome article pdf available in journal of child neurology 257. Clinicians need to be aware of vanishing bile duct syndrome as a serious consequence of ibuprofen use in adult patients, although ibuprofen is considered to. Vanishing bile duct syndrome and inflammatory pseudotumor. Vanishing bile duct syndrome refers to a group of acquired disorders resulting in progressive destruction and disappearance of the intrahepatic bile ducts and, ultimately, cholestasis. In children, biliary atresia and alagille syndrome are rare ductopenic diseases classified as vbds. Stevensjohnson syndrome and acute vanishing bile duct.
We present the case report of a 6yearold patient who developed stevensjohnson syndrome sjs and acute vanishing bile duct syndrome vbds after taking oral amoxicillin and naproxen. Vanishing bile duct syndrome definition of vanishing. Ibuprofen associated acute vanishing bile duct syndrome and toxic epidermal necrolysis have not been reported previously in infants. Lumley and elwyn eli as department of pathology, university of birmingham, birmingham b15 2tt. Most cases of vbds arise within a few months of onset of severe cholestatic hepatitis, often with immunoallergic features such as rash, fever. Request pdf vanishing bile duct syndrome perhaps no condition associated with chronic cholestasis is less understood than vanishing bile duct syndrome. Though its causes are unknown, an immunerelated pathogenesis is the most likely one. She developed significant cachexia and pruritus that did not respond to medical therapy, and hence she was listed for liver. Typically, this occurs as a progressive lesion resulting in irreversible damage and graft failure requiring retransplantation. Vanishing bile duct syndrome an overview sciencedirect topics. Sequential liver biopsies confirmed the development of vanishing bile duct syndrome. Vanishing bile duct syndrome with hyperlipidemia after ibuprofen therapy in an adult patient.
The current definition of ductopenia is the loss of. Complete remission of paraneoplastic vanishing bile duct. Vanishing bile duct syndrome associated with peripheral t cell lymphoma, not otherwise speci. Vanishing bile duct syndrome associated with chronic ebv infection digestive diseases and sciences, vol. Vanishing bile duct syndrome vbds is a rare form of liver injury and can be caused by multiple etiologies including malignancy. Vanishing bile duct syndrome has been associated with different pathologic conditions adverse drug reactions, autoimmune diseases, graft versus host disease, and cancer. Sjs, an immune complexmediated hypersensitivity reaction involving the skin and mucosa, is usually druginduced, and it can lead to systemic symptoms.
A case report of vanishing bile duct syndrome after exposure. Vanishing bile duct syndrome comprises a number of chronic cholestatic diseases caused by primary immunological and functional damage to the biliary epithelium. Vanishing bile duct syndrome definition of vanishing bile. Multiple causes have been convinced to be associated with vbds, including congenital and genetic diseases, neoplasms, infection, and drugs. The pathogenesis is unclear and related to immunologic injury of bile ducts. Recently, the vanishing bile duct syndrome was described in hodgkins disease.
Nonsteroidal antiinflammatory drugs nsaids are frequently prescribed drugs and can cause druginduced liver injury. Vanishing bile duct syndrome in druginduced liver disease. Vanishing bile duct syndrome vbds is a rare acquired condition, characterized by progressive loss of intrahepatic bile ducts, which is evidenced in liver biopsy, and leads to ductopenia and cholestasis. Vanishing bile duct syndrome as a manifestation of hodgkins. The current definition of ductopenia is the loss of interlobular bile ducts in more than 50% of portal tracts. It is therefore critical for physicians to create a broad differential when vbds is suspected and diagnosed. Department of haematology, east birmingham hospital, birmingham b9 5st. Vanishing bile duct syndrome with hemophagocytic lymphohistiocytosis after. Vanishing bile duct syndrome as a manifestation of hodgkin. Clinical findings fatigue, anorexia, right upper quadrant pain, malabsorption, weight loss, pruritis. Deeper insights into vanishing bile duct syndrome in.
Vanishing bile duct syndrome with eruptive xanthomas. Vanishing bile duct syndrome vbds is a rare and potentially life. Diagnosis at this point was intrahepatic cholestasis, requiring the condition vanishing bile duct syndrome vbds to be ruled out. In particular, vanishing bile duct syndrome is a progressive and always fatal complication in this setting, although some reversible cases have been described in association with other liver transplantations. Although the pathogenesis is poorly understood, vbds has been associated with potential infectious etiologies, ischemia, autoimmune diseases, adverse drug reactions, and humoral. The pathogenesis and possible significance of this lesion in the differential diagnosis of hdassociated cholestasis are considered further. Loss of bile ducts is a characteristic feature of chronic rejection in the liver allograft also known as irreversible rejection and vanishing bileduct syndrome. Any of a group of acquired hepatopathies characterised by irreversible destructive cholangitis, loss of intrahepatic bile ducts and chronic cholestasis. Vanishing bile duct syndrome can evolve to hepatic failure and, eventually, to death. Inspissated bile syndrome is defined as partial or complete obstruction of the extrahepatic biliary system by impaction of thick bile or sludge in the distal common bile duct during the neonatal period.
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