Evidence-based therapy of primary biliary cirrhosis

Ej, Heathcote

doi:10.1097/00042737-199906000-00005

Cited by 24 publications

(17 citation statements)

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“…Die PBC ist von ihrer Präsentation und ihren Charakteristika her eine typische Autoimmunerkrankung,sodass die meisten therapeutischen Ansätze mit immunsuppressiven Wirkstoffen unternommen wurden (⊡ Tabelle 5).Insgesamt ist die Immunsuppression bei der PBC nicht über-zeugend und mit einem deutlichen Nebenwirkungsprofil assoziiert, sodass sie nicht generell empfohlen werden kann [19]. Als Standardtherapie, die allerdings nur eine begrenzte lebensverlängernde Wirkung hat und anders als die Lebertransplantation keine Heilung der Erkrankung erreicht, gilt die Gabe von Ursodeoxycholsäure [42].…”

Section: Medikamentöse Behandlung Der Pbcunclassified

Prim�r bili�re Leberzirrhose und Overlap-Syndrome

Strassburg

Manns

2004

Der Internist

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Primary biliary cirrhosis represents a chronic cholestatic liver disease of unknown etiology. It primarily affects females, is associated with extrahepatic immune-mediated syndromes, shows an immunogenetic association with HLA DR8, and displays serum autoantibodies, which makes an autoimmune etiology likely. The diagnosis is reached in patients with elevated alkaline phosphatase, gamma glutamyl transferase and bilirubin levels who exhibit normal bile ducts upon ultrasound examination, and in whom specific antimitochondrial autoantibodies are detectable. Half of all PBC patients additionally show specific antinuclear autoantibodies. Immunosuppressive therapy is ineffective; steroids, transplant immunosuppressants, colchicine, d-penicillamine and methotrexate are of limited clinical benefit. Ursodeoxycholic acid has few side effects and leads to a biochemical response and a delay of disease progression in most cases. When ursodeoxycholic acid therapy is ineffective an overlap syndrome with autoimmune hepatitis can be present, which can respond to steroid treatment. The only curative option is liver transplantation which should be considered when bilirubin levels exceed 100 microM/l.

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Section: Medikamentöse Behandlung Der Pbcunclassified

Prim�r bili�re Leberzirrhose und Overlap-Syndrome

Strassburg

Manns

2004

Der Internist

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“…In all trials it was shown that even short term treatment with UDCA leads to a marked improvement in the serum markers of chronic cholestasis [28]. However, only 30 % of UDCA treated patients have a complete normalization of their biochemical tests, whereas 70% are incomplete responders [29].…”

Section: Effect On Laboratory Valuesmentioning

confidence: 99%

“…Because PBC appears to be an autoimmune disease, several immunosuppressive drugs have been tested in randomized controlled trials [28]. However, so far none has been shown to be of great benefit and their use is limited due to their side effects.…”

Section: Immunosuppressants In Primary Biliary Cirrhosismentioning

confidence: 99%

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Drug therapy of primary biliary diseases: classical and modern strategies

Schlichting

Leuschner

2001

J Cellular Molecular Medi

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“…Primary therapy for PBC has included many uncontrolled and controlled trials of various single regimens which can be itemized based on their mechanism of proposed function (Table 3) [5]. They display immunosuppressive, antiinflammatory, anti-fibrotic, choleretic, and cupruretic properties or a combination of these (Table 3).…”

Section: Primary Therapymentioning

confidence: 99%

Management of primary biliary cirrhosis: From diagnosis to end-stage disease

Lazaridis

Lindor

2000

Curr Gastroenterol Rep

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Primary biliary cirrhosis (PBC) is one of the most common chronic cholestatic liver diseases affecting the adult population. The clinical presentation of PBC can be diverse, ranging from the presymptomatic individual to the patient with advanced liver disease. Initial evaluation to establish diagnosis and appropriate follow-up are very important in the life-long management of these patients. The primary medical treatment in PBC should focus on reducing the rate of disease progression. To this extent, treatment with ursodeoxycholic acid has been extensively evaluated and has been shown to improve liver biochemistries and survival in patients with PBC. Secondary medical management in PBC should address the treatment of complications of chronic cholestasis, hepatic cirrhosis, and liver failure. Liver transplantation remains the only established therapeutic approach in treatment of patients with end-stage PBC and the associated complications.

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Evidence-based therapy of primary biliary cirrhosis

Cited by 24 publications

References 0 publications

Prim�r bili�re Leberzirrhose und Overlap-Syndrome

Prim�r bili�re Leberzirrhose und Overlap-Syndrome

Drug therapy of primary biliary diseases: classical and modern strategies

Management of primary biliary cirrhosis: From diagnosis to end-stage disease

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