Natural history of primary biliary cirrhosis

Parés, Albert; Rodés, Juan

doi:10.1016/s1089-3261(03)00100-4

Cited by 86 publications

(58 citation statements)

References 65 publications

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“…17 It is well-established that PBC has a genetic component that confers individual susceptibility. 5 Prior epidemiological and experimental data suggest that environmental factors might also play an important role in the pathogenesis of the disease, but the design of these studies and the size of the cohorts and case finding methods limited the degree of confidence in the results.…”

Section: Discussionmentioning

confidence: 99%

Risk Factors and Comorbidities in Primary Biliary Cirrhosis: A Controlled Interview-Based Study of 1032 Patients *

et al. 2005

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Section: Discussionmentioning

confidence: 99%

Risk Factors and Comorbidities in Primary Biliary Cirrhosis: A Controlled Interview-Based Study of 1032 Patients *

et al. 2005

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“…The rest will develop symptoms within the next 4 years. The commonest symptom at presentation is fatigue followed by pruritus (21% and 19% respectively) [204][205][206] .…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Primary biliary cirrhosis: From bench to bedside

Notas

2015

WJGPT

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Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis after a protractive non cirrhotic stage. The etiology and pathogenesis are largely unknown and autoimmne mechanisms have been implicated to explain the pathological lesions. Many epitopes and autoantigens have been reported as crucial in the pathophysiology of the disease and T and B cells abnormalities have been described, the exact pathways leading to the destruction of small intrahepatic ductules are mostly speculative. In this review we examined the various epidemiologal and geoepidemiological data as well as the complex pathogenetic aspects of this disease, focusing on recent in vivo and in vitro studies in this field. Initiation and progression of PBC is believed to be a multifactorial process with strong infuences from the patient's genetic background and by various environmental factors. The role of innate and adaptive immunity, including cytokines, chemokines, macrophages and the involvement of apoptosis and reactive oxygen species are outlined in detailed. The current pathogenetic aspects are presented and a novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.A review of clinical manifestations and immunological and pathological diagnosis was presented. Treatment modalities, including the multiple mechanisms of action of ursodeoxycholate were finally discussed. Core tip: Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis of largely unknown pathophysiology. We examined the epidemiological and geoepidemiological data as well as the pathogenetic aspects of PBC. A novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.

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“…This lack of predictive value may be due to the fact that the prolongation of prothrombin time in patients with cirrhosis occurs very late in the evolution of the disease. In other disease states such as primary biliary cirrhosis or primary sclerosing cholangitis bilirubin levels in conjunction with albumin are considered very good markers of prognosis [11].…”

Section: Liver Functionmentioning

confidence: 99%