Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis)

Ludwig, Jürgen; Dickson, E. Rolland; McDonald, G. S. A.

doi:10.1007/bf00432479

Cited by 710 publications

(382 citation statements)

References 4 publications

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“…Liver biopsies, obtained from patients with abnormal liver function tests and negative virology studies, with normal architecture, were used as normal controls. All patients with a first diagnosis of PBC were classified as stages II or III, according to Ludwig [10]. Patients with stage IV (cirrhosis) were excluded.…”

Section: Patientsmentioning

confidence: 99%

CXCR3 axis in patients with primary biliary cirrhosis: a possible novel mechanism of the effect of ursodeoxycholic acid

Manousou

Kolios

Drygiannakis

et al. 2013

Clinical and Experimental Immunology

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Section: Patientsmentioning

confidence: 99%

CXCR3 axis in patients with primary biliary cirrhosis: a possible novel mechanism of the effect of ursodeoxycholic acid

Manousou

Kolios

Drygiannakis

et al. 2013

Clinical and Experimental Immunology

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“…The presence of fibrous septa or bridging necrosis is classified as stage 3 and cirrhosis as stage 4. 26 The presence of fibrosis or cirrhosis does indicate a worse prognosis than if no fibrosis is seen on biopsy. 27 If the AMA is negative or in low titer (Ͻ1:40) or if the patient has a biochemical picture with prominent elevation of transaminase levels, i.e.…”

Section: Liver Biopsymentioning

confidence: 99%

Management of primary biliary cirrhosis

2000

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Primary biliary cirrhosis (PBC) is a presumed autoimmune disease of the liver, which predominantly affects women once over the age of 20 years. Most cases are diagnosed when asymptomatic (60%). The antimitochondrial antibody is present in serum in most, but not in all, patients with PBC. The disease generally progresses slowly but survival is less than an age-and gender-matched general population. The symptomatic patient may have fatigue, generalized pruritus, portal hypertension, osteoporosis, skin xanthomata, fat soluble vitamin deficiencies, and/or recurrent asymptomatic urinary tract infections. Many nonhepatic autoimmune diseases are found in association with PBC and may prompt initial presentation. To date, immunosuppressive therapy has not been shown to prolong survival in PBC. The hydrophilic bile acid, ursodeoxycholic acid (UDCA), has been shown when given in a dose of 13 to 15 mg/kg daily for up to 4 years to delay the time to liver transplantation or death. This therapy also causes a significant improvement of all the biochemical markers of cholestasis but has no beneficial effects on any of the symptoms or associated disorders. Treatment with UDCA does not obviate the need for liver transplantation. Therapies to prevent complications arising from malabsorption, portal hypertension, and/or osteoporosis are required as well. Good control of pruritus can be achieved in most patients. PBC is diagnosed with increasing frequency, but the agent(s) responsible for this slowly progressive destruction of the interlobular bile ducts remains elusive and hence a specific therapy remains unavailable. (HEPATOLOGY 2000;31:1005-1013.) PREAMBLE

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“…The liver pathology in the patient (Fig. 1B) was stage I PBC according to the histological changes described in the previous report (Ludwig et al 1978). Immuno histochemical staining showed that the liver tissues from PBC patients contained increased number of vimentin (mesen- chymal marker)-positive IBECs (Fig.…”

Section: Ibecs-emt In Livers Of Pbc Patientsmentioning

confidence: 52%

“…PBC was categorized into four phases based on the following histological changes: stage I, cholangiolitis; stage II, bile duct proliferation; stage III, cicatrization; and stage IV, liver cirrhosis (Ludwig et al 1978). Serum samples were obtained from 29 PBC patients (2 males and 27 females; 19-80 years of age, mean age: 51 years, course of disease: 10 day to 315 month, average course: 40.69 months), and 11 healthy volunteers (2 males and 9 females; 29-64 years of age, mean age: 46.8 years) from July 2014 to September 2015.…”

Section: Patientsmentioning

confidence: 99%

Interleukin-17A-Induced Epithelial-Mesenchymal Transition of Human Intrahepatic Biliary Epithelial Cells: Implications for Primary Biliary Cirrhosis

Huang

Chu

Yin

et al. 2016

Tohoku J. Exp. Med.

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Primary biliary cirrhosis (PBC) is an autoimmune chronic liver disease with worldwide increasing morbidity. However, the etiology of PBC is still unclear. Recently, the epithelial-mesenchymal transition (EMT) and interleukin-17A (IL-17A), a pro-inflammatory cytokine, were proposed to be involved in the pathogenesis of PBC. Therefore, in this study, we aimed to clarify the roles of IL-17A and/or EMT in the onset of PBC. The results showed that the median serum IL-17A level was significantly higher in 29 PBC patients (average course of 40.69 months) than that of 11 healthy controls. The intrahepatic biliary epithelial cells (IBECs), the major target of destruction in PBC, underwent EMT in PBC patients. The immunohistochemical analysis revealed that the protein levels of IL-17A receptor were increased in IBECs and the IL-17A protein was accumulated around the IBECs in the PBC patients. These results imply that the IL-17A-mediated signaling and EMT of intrahepatic biliary epithelial cells (IBEC-EMT) are key pathogenic processes of PBC. To study the association between IL-17A and IBECs-EMT, we then examined if IL-17A induced EMT using a human cell line of IBECs (HIBECs). After the treatment with IL-17A for 48 h, HIBECs changed into bipolar cells with a fibroblastic morphology. Additionally, the results of real-time PCR and Western blot analyses demonstrated that IL-17A up-regulated the expression of a mesenchymal marker vimentin and downregulated the expression of an epithelial marker E-cadherin in HIBECs in the dose-and time-dependent manners. These results suggest that IL-17A may play an important role in the IBECs-EMT.

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Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis)

Cited by 710 publications

References 4 publications

CXCR3 axis in patients with primary biliary cirrhosis: a possible novel mechanism of the effect of ursodeoxycholic acid

CXCR3 axis in patients with primary biliary cirrhosis: a possible novel mechanism of the effect of ursodeoxycholic acid

Management of primary biliary cirrhosis

Interleukin-17A-Induced Epithelial-Mesenchymal Transition of Human Intrahepatic Biliary Epithelial Cells: Implications for Primary Biliary Cirrhosis

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