Fatigue and pruritus at onset identify a more aggressive subset of primary biliary cirrhosis

Quarneti, Chiara; Muratori, Paolo; Lalanne, Claudine; Fabbri, Angelo; Menichella, R.; Granito, Alessandro; Masi, C; Lenzi, Marco; Cassani, F.; Pappas, Georgios

doi:10.1111/liv.12560

Cited by 65 publications

(37 citation statements)

References 23 publications

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“…Patients with PBC normally suffer from itching and fatigue, regardless of disease severity[14,37,38]. Serum fat-soluble vitamin D deficiency may be detected, particularly in advanced PBC patients[39,40].…”

Section: Risk Factorsmentioning

confidence: 99%

“…As the symptom of fatigue is non-specific, multifactorial, and potentially incapacitating, conditions such as anemia, diabetes, hypothyroidism, and depression should be considered and excluded[9-11]. Fatigue is typically identified with a subset of PBC patients who are predominantly young women who have particularly active illness, a suboptimal response to UDCA therapy, and are more likely to develop hepatic cirrhosis and its complications[37]. At present, there is no special drug therapy for the management of PBC-related fatigue and no significant improvement following liver transplantation[9-11].…”

Section: Risk Factorsmentioning

confidence: 99%

“…More than two-thirds of PBC patients experience pruritus during the process of the illness[9-11]. Compared to asymptomatic PBC patients without pruritus, symptomatic PBC patients with pruritus more frequently suffer from hepatic cirrhosis and its related complications ( P = 0.004)[37], and are less likely to respond to UDCA treatment ( P = 0.006). The pathogenesis of cholestatic pruritus remains largely elusive[9-11]; its natural history, related pathogenesis, and molecular mechanisms are under continued investigation[9-11].…”

Section: Risk Factorsmentioning

confidence: 99%

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Recent advances in the diagnosis and treatment of primary biliary cholangitis

Huang¹

2016

WJH

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Primary biliary cholangitis (PBC), formerly referred to as primary biliary cirrhosis, is an infrequent progressive intrahepatic cholestatic autoimmune illness that can evolve into hepatic fibrosis, hepatic cirrhosis, hepatic failure, and, in some cases, hepatocellular carcinoma. The disease itself is characterized by T-lymphocyte-mediated chronic non-suppurative destructive cholangitis and elevated serum levels of extremely specific anti-mitochondrial autoantibodies (AMAs). In this article, we will not only review epidemiology, risk factors, natural history, predictive scores, radiologic approaches (e.g., acoustic radiation force impulse imaging, vibration controlled transient elastography, and magnetic resonance elastography), clinical features, serological characteristics covering biochemical markers, immunoglobulins, infections markers, biomarkers, predictive fibrosis marker, specific antibodies (including AMAs such as AMA-M2), anti-nuclear autoantibodies [such as anti-multiple nuclear dot autoantibodies (anti-sp100, PML, NDP52, anti-sp140), anti-rim-like/membranous anti-nuclear autoantibodies (anti-gp210, anti-p62), anti-centromere autoantibodies, and some of the novel autoantibodies], histopathological characteristics of PBC, diagnostic advances, and anti-diastole of PBC. Furthermore, this review emphasizes the recent advances in research of PBC in terms of therapies, including ursodeoxycholic acid, budesonide, methotrexate, obeticholic acid, cyclosporine A, fibrates such as bezafibrate and fenofibrate, rituximab, mesenchymal stem cells transplant, and hepatic transplant. Currently, hepatic transplant remains the only optimal choice with acknowledged treatment efficiency for end-stage PBC patients.

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Section: Risk Factorsmentioning

confidence: 99%

Section: Risk Factorsmentioning

confidence: 99%

Section: Risk Factorsmentioning

confidence: 99%

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Recent advances in the diagnosis and treatment of primary biliary cholangitis

Huang¹

2016

WJH

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“…The prognostic importance of fatigue in PBC is contentious, but concern is perhaps best highlighted in the prospective cohort study from Jones et al (n = 136), wherein transplant‐free survival (TFS) was significantly shorter among fatigued patients relative to nonfatigued, disease‐matched controls (56% vs. 74%; P < 0.0001), independent of UDCA provision. Although a consensus biological explanation for fatigue is lacking, presenting age and sex heavily influence the clinical phenotype, with young women (a group failing UDCA therapy more commonly) having the greatest symptom burden . However, there is no evidence that symptomatic presentations impart additional discriminatory value to existing risk‐prediction models.…”

Section: Clinical History and Phenotypesmentioning

confidence: 99%

Risk stratification in autoimmune cholestatic liver diseases: Opportunities for clinicians and trialists

et al. 2015

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Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are infrequent autoimmune cholestatic liver diseases, that disproportionate to their incidence and prevalence, remain very important causes of morbidity and mortality for patients with liver disease. Mechanistic insights spanning genetic risks and biological pathways to liver injury and fibrosis have led to a renewed interest in developing therapies beyond ursodeoxycholic acid that are aimed at both slowing disease course and improving quality of life. International cohort studies have facilitated a much greater understanding of disease heterogeneity, and in so doing highlight the opportunity to provide patients with a more individualized assessment of their risk of progressive liver disease, based on clinical, laboratory, or imaging findings. This has led to a new approach to patient care that focuses on risk stratification (both high and low risk); and furthermore allows such stratification tools to help identify patient subgroups at greatest potential benefit from inclusion in clinical trials. In this article, we review the applicability and validity of risk stratification in autoimmune cholestatic liver disease, highlighting strengths and weaknesses of current and emergent approaches. (Hepatology 2016;63:644–659)

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“…36, Patients who present with pruritus early have poorer response to UDCA and a worse clinical outcome. 37 Budesonide, a non-halogenated glucocorticoid absorbed in the small bowel with a high first pass metabolism has been tried in PBC with encouraging results in pre-cirrhotic PBC as an add-on to UDCA. However, late stage PBC patients showed significant increase in budesonide plasma levels and developed serious side effects.…”

Section: Correct Answers: a C And Dmentioning

confidence: 99%