Primary biliary cirrhosis

Definition

Primary biliary cirrhosis is an autoimmune disease of the liver characterized by slow, progressive destruction of bile ducts in the liver. The early destruction of small interlobular bile ducts leads to build up of bile in the liver – cholestasis – tissue damage and, in some patients, cirrhosis. Common symptoms are fatigue and pruritus.

Latest Research and Reviews

  • Research | | open

    Primary biliary cholangitis is an autoimmune liver disease. Here, the authors show that variants in interleukin genes which potentially deregulate their expression are associated with this condition, and suggest that the IL21 signalling pathway may have a role in disease aetiology.

    • Fang Qiu
    • , Ruqi Tang
    • , Xianbo Zuo
    • , Xingjuan Shi
    • , Yiran Wei
    • , Xiaodong Zheng
    • , Yaping Dai
    • , Yuhua Gong
    • , Lan Wang
    • , Ping Xu
    • , Xiang Zhu
    • , Jian Wu
    • , Chongxu Han
    • , Yueqiu Gao
    • , Kui Zhang
    • , Yuzhang Jiang
    • , Jianbo Zhou
    • , Youlin Shao
    • , Zhigang Hu
    • , Ye Tian
    • , Haiyan Zhang
    • , Na Dai
    • , Lei Liu
    • , Xudong Wu
    • , Weifeng Zhao
    • , Xiaomin Zhang
    • , Zhidong Zang
    • , Jinshan Nie
    • , Weihao Sun
    • , Yi Zhao
    • , Yuan Mao
    • , Po Jiang
    • , Hualiang Ji
    • , Qing Dong
    • , Junming Li
    • , Zhenzhong Li
    • , Xinli Bai
    • , Li Li
    • , Maosong Lin
    • , Ming Dong
    • , Jinxin Li
    • , Ping Zhu
    • , Chan Wang
    • , Yanqiu Zhang
    • , Peng Jiang
    • , Yujue Wang
    • , Rohil Jawed
    • , Jing Xu
    • , Yu Zhang
    • , Qixia Wang
    • , Yue Yang
    • , Fan Yang
    • , Min Lian
    • , Xiang Jiang
    • , Xiao Xiao
    • , Yanmei Li
    • , Jingyuan Fang
    • , Dekai Qiu
    • , Zhen Zhu
    • , Hong Qiu
    • , Jianqiong Zhang
    • , Wenyan Tian
    • , Sufang Chen
    • , Ling Jiang
    • , Bing Ji
    • , Ping Li
    • , Guochang Chen
    • , Tianxue Wu
    • , Yan Sun
    • , Jianjiang Yu
    • , Huijun Tang
    • , Michun He
    • , Min Xia
    • , Hao Pei
    • , Lihua Huang
    • , Zhuye Qing
    • , Jianfang Wu
    • , Qinghai Huang
    • , Junhai Han
    • , Wei Xie
    • , Zhongsheng Sun
    • , Jian Guo
    • , Gengsheng He
    • , M. Eric Gershwin
    • , Zhexiong Lian
    • , Xiang Liu
    • , Michael F. Seldin
    • , Xiangdong Liu
    • , Weichang Chen
    •  & Xiong Ma
  • Reviews |

    Primary biliary cirrhosis (PBC) leads to progressive cholestasis, biliary fibrosis and cirrhosis and characteristic symptoms with a marked effect on quality of life. Evolution in our understanding of disease mechanisms in PBC is rapidly leading to the advent of new and re-purposed therapeutic agents targeting key processes. This Review provides a current perspective on potential approaches to PBC treatment, and highlights the challenges faced in evaluating and implementing those treatments.

    • Jessica K. Dyson
    • , Gideon M. Hirschfield
    • , David H. Adams
    • , Ulrich Beuers
    • , Derek A. Mann
    • , Keith D. Lindor
    •  & David E. J. Jones
  • Research | | open

    Primary biliary cirrhosis is an autoimmune liver disease with poor therapeutic options. Here Cordell et al. a perform meta-analysis of European genome-wide association studies identifying six novel risk loci and a number of potential therapeutic pathways.

    • Heather J. Cordell
    • , Younghun Han
    • , George F. Mells
    • , Yafang Li
    • , Gideon M. Hirschfield
    • , Casey S. Greene
    • , Gang Xie
    • , Brian D. Juran
    • , Dakai Zhu
    • , David C. Qian
    • , James A. B. Floyd
    • , Katherine I. Morley
    • , Daniele Prati
    • , Ana Lleo
    • , Daniele Cusi
    • , Canadian-US PBC Consortium
    • , Erik M Schlicht
    • , Craig Lammert
    • , Elizabeth J Atkinson
    • , Landon L Chan
    • , Mariza de Andrade
    • , Tobias Balschun
    • , Andrew L Mason
    • , Robert P Myers
    • , Jinyi Zhang
    • , Piotr Milkiewicz
    • , Jia Qu
    • , Joseph A Odin
    • , Velimir A Luketic
    • , Bruce R Bacon
    • , Henry C Bodenheimer Jr
    • , Valentina Liakina
    • , Catherine Vincent
    • , Cynthia Levy
    • , Peter K Gregersen
    • , Italian PBC Genetics Study Group
    • , Piero L Almasio
    • , Domenico Alvaro
    • , Pietro Andreone
    • , Angelo Andriulli
    • , Cristina Barlassina
    • , Pier Maria Battezzati
    • , Antonio Benedetti
    • , Francesca Bernuzzi
    • , Ilaria Bianchi
    • , Maria Consiglia Bragazzi
    • , Maurizia Brunetto
    • , Savino Bruno
    • , Giovanni Casella
    • , Barbara Coco
    • , Agostino Colli
    • , Massimo Colombo
    • , Silvia Colombo
    • , Carmela Cursaro
    • , Lory Saveria Crocè
    • , Andrea Crosignani
    • , Maria Francesca Donato
    • , Gianfranco Elia
    • , Luca Fabris
    • , Carlo Ferrari
    • , Annarosa Floreani
    • , Barbara Foglieni
    • , Rosanna Fontana
    • , Andrea Galli
    • , Roberta Lazzari
    • , Fabio Macaluso
    • , Federica Malinverno
    • , Fabio Marra
    • , Marco Marzioni
    • , Alberto Mattalia
    • , Renzo Montanari
    • , Lorenzo Morini
    • , Filomena Morisco
    • , Mousa Hani S
    • , Luigi Muratori
    • , Paolo Muratori
    • , Grazia A Niro
    • , Vincenzo O Palmieri
    • , Antonio Picciotto
    • , Mauro Podda
    • , Piero Portincasa
    • , Vincenzo Ronca
    • , Floriano Rosina
    • , Sonia Rossi
    • , Ilaria Sogno
    • , Giancarlo Spinzi
    • , Marta Spreafico
    • , Mario Strazzabosco
    • , Sonia Tarallo
    • , Mirko Tarocchi
    • , Claudio Tiribelli
    • , Pierluigi Toniutto
    • , Maria Vinci
    • , Massimo Zuin
    • , UK-PBC Consortium
    • , Chin Lye Ch'ng
    • , Mesbah Rahman
    • , Tom Yapp
    • , Richard Sturgess
    • , Christopher Healey
    • , Sushma Saksena
    • , Toby Delahooke
    • , Maxton Pitcher
    • , Phillipa Desousa
    • , David Elphick
    • , Markus Gess
    • , Suzie Marriott
    • , Alistair King
    • , Susan Jones
    • , Andrew Burroughs
    • , Andrew Austin
    • , Lourdes Cumlat
    • , Mark Aldersley
    • , Janine Musselwhite
    • , Rose Damant
    • , Kate Ocker
    • , Andrew Douds
    • , Emily Archer
    • , Valeria Silvestre
    • , Oza Chirag
    • , Anton Gunasekera
    • , Marco Carbone
    • , Linda Kent
    • , Carol Thomas
    • , Jacky Orpe
    • , Harriet Gordon
    • , Earl Williams
    • , Jennie Kendall
    • , Rebecca Casey
    • , Hyder Hussaini
    • , Jessica Whiteman
    • , Emma Grieve
    • , Aditya Mandal
    • , Roger McCorry
    • , Charles Grimley
    • , Keith George
    • , Jane Bowles
    • , Lucie Hobson
    • , Kathryn Houghton
    • , Graham Foster
    • , Kapil Kapur
    • , Mary Crossey
    • , Sharat Misra
    • , Lyn Graves
    • , Simon Taylor-Robinson
    • , Sue Glenn
    • , Sandra Greer
    • , PuiChing Chan
    • , Syed Shaukat
    • , Konrad Koss
    • , Helen Wooldridge
    • , Linda Jones
    • , Abigail Hynes
    • , Christopher I. Amos
    • , James Featherstone
    • , Rebecca Cloudsdale
    • , Ian Rees
    • , Nikki White
    • , Joy Sadeghian
    • , Jean Kordula
    • , Andrea Broad
    • , Andrew Bell
    • , Graeme Alexander
    • , Marisa Mills
    • , Talal Valliani
    • , Janette Curtis
    • , Caroline Cowley
    • , Sue Walsh
    • , Harriet Mitchison
    • , Peter Bramley
    • , Donna Cotterill
    • , Jacqueline Brighton
    • , Jeremy Tibble
    • , Amit Singhal
    • , Stewart Campbell
    • , Tom Lee
    • , Reuben Ayres
    • , Javaid Subhani
    • , Alastair McNair
    • , Carl A. Anderson
    • , Jonathan Booth
    • , Voi Shim Wong
    • , Kelly Amor
    • , Jonathan Brown
    • , Ian Gee
    • , Elva Wilhelmsen
    • , Rebecca Bishop
    • , James Gotto
    • , Jane Collier
    • , Caroline Lawlor
    • , Lola Dali-kemmery
    • , Senayon Desmennu
    • , Parizade Raymode
    • , Sherill Tripoli
    • , Kate Martin
    • , Mike Mendall
    • , Ian Drake
    • , Bronwen Williams
    • , Steve Pereira
    • , Keith Elliott
    • , Juliette Verheyden
    • , Esther Unitt
    • , Guan Lim
    • , Darren Clement
    • , Roland Ede
    • , Victoria Lambourne
    • , Douglas Thorburn
    • , Mark Cox
    • , Ervin Shpuza
    • , Andrew Fraser
    • , Katharine Pollock
    • , David Gorard
    • , Helen Fairlamb
    • , Neil Fisher
    • , John Mclindon
    • , Yeng Ang
    • , Graham Butcher
    • , Kerry Flahive
    • , Saket Singhal
    • , Helen Doyle
    • , John Ramage
    • , Karin Young
    • , Natalie Wheatley
    • , Marina Leoni
    • , Julia Maltby
    • , Helen Sweeting
    • , Julie Tregonning
    • , Anna Fouracres
    • , Greta Van Duyvenvoorde
    • , Carolyn MacNicol
    • , Simon Rushbrook
    • , Daniel Forton
    • , Liz Farrington
    • , Gordon Wood
    • , Julie Colley
    • , Kevin Yoong
    • , Frances Hines
    • , Howard Curtis
    • , Andrew Gibbins
    • , Katie Seward
    • , Mark Wright
    • , Richard Marley
    • , Purushothaman Premchand
    • , Gill Watts
    • , Isobel Amey
    • , Hannah Dewhurst
    • , Subramaniam Ramakrishnan
    • , Jocelyn Fraser
    • , Deb Ghosh
    • , Ian Gooding
    • , Chris Ford
    • , Alan Watson
    • , Jennifer Morrison
    • , M. Eric Gershwin
    • , Fiona Gordon
    • , Tim Harding
    • , George Bird
    • , Mark Wilkinson
    • , Ashley Brown
    • , Mary Ninkovic
    • , Mandy Carnahan
    • , Paul Kitchen
    • , Sanjiv Jain
    • , Eleanor Dungca
    • , Natalie Taylor
    • , Jill Conder
    • , Mcdonald Mupudzi
    • , Sophie Holder
    • , David Jones
    • , Katherine A. Siminovitch
    • , Gregory Whatley
    • , LaiTing Tan
    • , Catherine Gray
    • , Kerenza Boulton
    • , Caron Innes
    • , Melanie Harrison
    • , Samantha Ducker
    • , Richard N. Sandford
    • , Ruth Penn
    • , George MacFaul
    • , Sarah Jones
    • , Lynn O'Donohoe
    • , Pietro Invernizzi
    • , Andrew Douglass
    • , Kirsten Walker
    • , James Neuberger
    • , Bal Shinder
    • , Theodore Ngatchu
    • , Gwen Baxter
    • , Jane Gitahi
    • , Faiz Ali
    • , Sarah Besley
    • , Imroz Salam
    • , Susan Slininger
    • , Matthew Cowan
    • , Maria Hamilton
    • , Caroline Hamilton
    • , Lorraine Hankey
    • , Andrzej Piotrowicz
    • , Florin Muscariu
    • , Charles Millson
    • , Eileen Marshall
    • , Sally Batham
    • , Leonie Grellier
    • , Michael F. Seldin
    • , Carly Brown
    • , Alison Paton
    • , Lynn Currie
    • , Steven Mann
    • , Hemant Gupta
    • , Diane Simpson
    • , Rupert Ransford
    • , Matthew Foxton
    • , Beverley Longhurst
    • , Claire Duggan
    • , Kimberley Netherton
    • , George Lipscomb
    • , Debashis Das
    • , Imran Patanwala
    • , Marie Quinn
    • , Sambit Sen
    • , Alison Loftus
    • , Hye-Jeong Lee
    • , Chris Evans
    • , Martin Prince
    • , Janeane Hails
    • , Barbara Hoeroldt
    • , Elizabeth Tanqueray
    • , Susan Lord
    • , Fiona Wright
    • , Christopher Hovell
    • , William Stableforth
    • , Janie Keggans
    • , Nicholas Sharer
    • , Christopher Macdonald
    • , John Dillon
    • , Alice Wright
    • , Alan Shepherd
    • , Joanna Sayer
    • , Adrian Barnardo
    • , Louise Winter
    • , Emma Durant
    • , Paul Southern
    • , Matthew Brookes
    • , Jeremy Shearman
    • , Susan Gallagher
    • , Jeff Butterworth
    • , Raj Srirajaskanthan
    • , Carole Foale
    • , Lizzie Stafford
    • , Simon Williams
    • , Amir Shah
    • , Sass Levi
    • , Howard Klass
    • , George Abouda
    • , Maria Hayes
    • , Andrew Chilton
    • , Geeta Prasad
    • , Mazn Karmo
    • , Sally-Ann Rolls
    • , Julie Browning
    • , Richard Miller
    • , Delyth Braim
    • , Denise O'Donnell
    • , Andrew Higham
    • , Anne Eastick
    • , Denise Gocher
    • , Caradog Thomas
    • , Bob Grover
    • , Jo Ridpath
    • , Jaber Gasem
    • , Pradeep Sanghi
    • , Nurani Sivaramakrishnan
    • , Jane Metcalf
    • , Paula Townshend
    • , Sallyanne Pearson
    • , Andrew Davis
    • , Sarah Thornthwaite
    • , Glynis Stansfield
    • , Rebecca Jones
    • , Metod Oblak
    • , Hilary Pateman
    • , Marie Green
    • , Lisa Morgan
    • , Andrew Bathgate
    • , Kirsty Martin
    • , Minesh Patel
    • , Paul Richardson
    • , David Tyrer
    • , Roman Galaska
    • , Peter Mills
    • , Becky Holbrook
    • , Susan Congreave
    • , Gayle Clifford
    • , Karen Hogben
    • , Ray Mathew
    • , Aruna Dias
    • , Emma Gunter
    • , Martin Lombard
    • , Patrick Goggin
    • , Caroline Palmer
    • , Jayne Jones
    • , Tracey Shewan
    • , Carole Collins
    • , Alexandra McKay
    • , Gary Bray
    • , Katie Spurdle
    • , David Ramanaden
    • , Linda March
    • , Martyn Carter
    • , Allister Grant
    • , Aftab Ala
    • , Samir Vyas
    • , Melanie Kent
    • , Ray Shidrawi
    • , Maggie Hughes
    • , Paul Banim
    • , Altaf Palegwala
    • , Joy Wilkins
    • , Sunil Dolwani
    • , Simon Whalley
    • , Noma Ncube
    • , Bridget Gunson
    • , Athar Saeed
    • , David Neal
    • , Rachel Abrahams
    • , Karen Warner
    • , Thiriloganathan Mathialahan
    • , Stephen D Ryder
    • , Christine Dickson
    • , Udi Shmueli
    • , Marek Czajkowski
    • , Pranab Gyawali
    • , Dermot Gleeson
    • , Christopher Shorrock
    • , Richard Aspinall
    • , Mark Ainsworth
    • , Mark Narain
    • , Ashis Mukhopadhya
    • , Simon Panter
    • , Nicola Lancaster
    • , Mervyn Davies
    • , Rory Harvey
    • , Mariam Nasseri
    • , Asifabbas Naqvi
    • , Matthew Cramp
    • , Michael Heneghan
    • , Richard Evans
    • , Estelle Nambela
    • , Jane Maiden
    • , Subrata Saha
    • , Andy Li
    • , Konstantinos N. Lazaridis
    • , Caroline Mulvaney-Jones
    • , Bridget Bird
    • , Christopher Shallcross
    • , D Williams
    • , Mohamed Dawwas
    • , Joanna Allison
    • , Dawn Sirdefield
    • , Simon Dyer
    • , Alison Brind
    • , Robert Przemioslo
    •  & Yolanda Baird

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