<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1130-0108</journal-id>
<journal-title><![CDATA[Revista Española de Enfermedades Digestivas]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. esp. enferm. dig.]]></abbrev-journal-title>
<issn>1130-0108</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Española de Patología Digestiva]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1130-01082018001000007</article-id>
<article-id pub-id-type="doi">10.17235/reed.2018.5665/2018</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Colangitis biliar primaria en España. Resultados de un estudio Delphi sobre su epidemiología, diagnóstico, seguimiento y tratamiento]]></article-title>
<article-title xml:lang="en"><![CDATA[Primary biliary cholangitis in Spain. Results of a Delphi study of epidemiology, diagnosis, follow-up and treatment]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Parés]]></surname>
<given-names><![CDATA[Albert]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Albillos]]></surname>
<given-names><![CDATA[Agustín]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Andrade]]></surname>
<given-names><![CDATA[Raúl-J.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Berenguer]]></surname>
<given-names><![CDATA[Marina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Crespo]]></surname>
<given-names><![CDATA[Javier]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Romero-Gómez]]></surname>
<given-names><![CDATA[Manuel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vergara]]></surname>
<given-names><![CDATA[Mercè]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vendrell]]></surname>
<given-names><![CDATA[Belén]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gil]]></surname>
<given-names><![CDATA[Alicia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de Barcelona Hospital Clínic. IDIBAPS, CIBERehd Unidad de Hepatología]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad de Alcalá Hospital Universitario Ramón y Cajal. IRYCIS, CIBERehd Servicio de Gastroenterología y Hepatología]]></institution>
<addr-line><![CDATA[Madrid ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Complejo Hospitalario de Especialidades Virgen de la Victoria Unidad de Aparato Digestivo ]]></institution>
<addr-line><![CDATA[Málaga ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Universidad de Valencia. CIBEREHD Hospital Universitari i Politècnic la Fe Servicio de Aparato Digestivo]]></institution>
<addr-line><![CDATA[Valencia ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Hospital Universitario Marqués de Valdecilla Servicio de Aparato Digestivo ]]></institution>
<addr-line><![CDATA[Santander ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,Hospital Virgen del Rocío UGC de Aparato Digestivo ]]></institution>
<addr-line><![CDATA[Sevilla ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af7">
<institution><![CDATA[,Universitat Autònoma de Barcelona Institut d'Investigació i Innovació Parc Taulí I3PT Unidad de Hepatología. Servicio de Aparato Digestivo. Parc Taulí Sabadell]]></institution>
<addr-line><![CDATA[Sabadell, Barcelona ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af8">
<institution><![CDATA[,Intercept Pharmaceuticals Departamento Médico ]]></institution>
<addr-line><![CDATA[Madrid ]]></addr-line>
<country>Spain</country>
</aff>
<aff id="Af9">
<institution><![CDATA[,Omakase Consulting Departamento Market Access ]]></institution>
<addr-line><![CDATA[Barcelona ]]></addr-line>
<country>Spain</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>10</month>
<year>2018</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>10</month>
<year>2018</year>
</pub-date>
<volume>110</volume>
<numero>10</numero>
<fpage>641</fpage>
<lpage>649</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_arttext&amp;pid=S1130-01082018001000007&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_abstract&amp;pid=S1130-01082018001000007&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.isciii.es/scielo.php?script=sci_pdf&amp;pid=S1130-01082018001000007&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  la colangitis biliar primaria (CBP) es una enfermedad rara, de la que existe información limitada en España sobre su epidemiología y manejo en la práctica clínica habitual.  Objetivos:  conocer la epidemiología, flujo del paciente, diagnóstico, seguimiento y tratamiento de la CBP en España.  Métodos:  revisión de la literatura y estudio siguiendo la metodología Delphi con participación de 28 especialistas en dos rondas de consulta y un taller de validación de resultados presencial.  Resultados:  existen, aproximadamente, 9.400 pacientes con CBP en España, con una incidencia anual de entre 0,51 y 3,86 casos/100.000 habitantes, aunque el margen de error se presupone alto dada la escasez de estudios. El intervalo entre sospecha y confirmación diagnóstica puede ser de varios meses, realizándola mayoritariamente un gastroenterólogo o hepatólogo. El papel de la biopsia hepática en el diagnóstico y seguimiento es heterogéneo. El 95% de los pacientes son tratados con ácido ursodesoxicólico (AUDC) y la respuesta se monitoriza mayoritariamente utilizando el criterio de Barcelona. El seguimiento es semestral, con un uso heterogéneo de las diferentes técnicas disponibles. No existen recomendaciones ni fármacos comercializados en segunda línea en caso de no respuesta, respuesta insuficiente o intolerancia al tratamiento con AUDC.  Conclusiones:  aunque es posible estimar la epidemiología a partir de la opinión de expertos, se necesitan registros nacionales que aporten información precisa y actualizada sobre la epidemiología, el estadio y la respuesta al tratamiento de los pacientes con CBP, así como tratamientos nuevos para ciertos grupos de pacientes.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  primary biliary cholangitis (PBC) is a rare disease with limited data regarding its epidemiology and standard clinical management in Spain.  Objective:  to gain insight into the epidemiology, patient flow, diagnosis, follow-up and treatment of PBC in Spain.  Methods:  a review of the literature and Delphi study involving 28 specialists in two rounds of consultations and an in-person results validation workshop.  Results:  there are approximately 9,400 patients with PBC in Spain, with an annual incidence of 0.51-3.86 cases/100,000 population. Albeit, a high error margin may be presumed due to the scarcity of relevant studies on this subject. Several months may elapse from suspicion to a confirmed diagnosis, usually by a gastroenterologist or hepatologist. The role of the liver biopsy for diagnosis and follow-up is heterogeneous. Overall, 95% of patients are treated with ursodeoxycholic acid (UDCA) and response is primarily monitored using the Barcelona criteria. Follow-up is performed every six months, with a heterogeneous use of the various available techniques. No recommendations or second-line commercial drugs are available in the case of no response, inadequate response or intolerance to UDCA.  Conclusions:  while epidemiology may be estimated based on expert opinions, national registries are needed to provide accurate, up-to-date information on epidemiological parameters, disease stage and response to treatment in patients with PBC. Furthermore, novel therapies are required for selected patient groups.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Colangitis biliar primaria]]></kwd>
<kwd lng="es"><![CDATA[Epidemiología]]></kwd>
<kwd lng="es"><![CDATA[Diagnóstico]]></kwd>
<kwd lng="es"><![CDATA[Tratamiento]]></kwd>
<kwd lng="en"><![CDATA[Primary biliary cholangitis]]></kwd>
<kwd lng="en"><![CDATA[Epidemiology]]></kwd>
<kwd lng="en"><![CDATA[Diagnosis]]></kwd>
<kwd lng="en"><![CDATA[Treatment]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[The diagnosis of primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bowlus]]></surname>
<given-names><![CDATA[CL]]></given-names>
</name>
<name>
<surname><![CDATA[Gershwin]]></surname>
<given-names><![CDATA[ME]]></given-names>
</name>
</person-group>
<source><![CDATA[Autoimmun Rev]]></source>
<year>2014</year>
<volume>13</volume>
<numero>4-5</numero>
<issue>4-5</issue>
<page-range>441-4</page-range></nlm-citation>
</ref>
<ref id="B2">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Treatment of primary biliary cirrhosis is there more to offer than ursodeoxycholic acid?]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Parés]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<source><![CDATA[Clin Liver Dis]]></source>
<year>2014</year>
<volume>3</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>29-33</page-range></nlm-citation>
</ref>
<ref id="B3">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Nguyen]]></surname>
<given-names><![CDATA[DL]]></given-names>
</name>
<name>
<surname><![CDATA[Juran]]></surname>
<given-names><![CDATA[BD]]></given-names>
</name>
<name>
<surname><![CDATA[Lazaridis]]></surname>
<given-names><![CDATA[KN]]></given-names>
</name>
</person-group>
<source><![CDATA[Best Pract Res Clin Gastroenterol]]></source>
<year>2010</year>
<volume>24</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>647-54</page-range></nlm-citation>
</ref>
<ref id="B4">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Demographic, lifestyle, medical and familial factors associated with primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Corpechot]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Chrétien]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Chazouillères]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<source><![CDATA[J Hepatol]]></source>
<year>2010</year>
<volume>53</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>162-9</page-range></nlm-citation>
</ref>
<ref id="B5">
<nlm-citation citation-type="">
<collab>Orphanet Report Series</collab>
<source><![CDATA[Prevalencia de las enfermedades raras: datos bibliográficos enfermedades listadas por orden de prevalencia o incidencia decreciente o por número de casos publicados]]></source>
<year>2016</year>
</nlm-citation>
</ref>
<ref id="B6">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Environmental factors in primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Juran]]></surname>
<given-names><![CDATA[BD]]></given-names>
</name>
<name>
<surname><![CDATA[Lazaridis]]></surname>
<given-names><![CDATA[KN]]></given-names>
</name>
</person-group>
<source><![CDATA[Semin Liver Dis]]></source>
<year>2014</year>
<volume>34</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>265-72</page-range></nlm-citation>
</ref>
<ref id="B7">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kumagi]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Heathcote]]></surname>
<given-names><![CDATA[EJ]]></given-names>
</name>
</person-group>
<source><![CDATA[Orphanet J Rare Dis]]></source>
<year>2008</year>
<volume>3</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>1</page-range></nlm-citation>
</ref>
<ref id="B8">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[The natural history of asymptomatic primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kurtovic]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Riordan]]></surname>
<given-names><![CDATA[SM]]></given-names>
</name>
<name>
<surname><![CDATA[Williams]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<source><![CDATA[QJM]]></source>
<year>2005</year>
<volume>98</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>331-6</page-range></nlm-citation>
</ref>
<ref id="B9">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lindor]]></surname>
<given-names><![CDATA[KD]]></given-names>
</name>
<name>
<surname><![CDATA[Gershwin]]></surname>
<given-names><![CDATA[ME]]></given-names>
</name>
<name>
<surname><![CDATA[Poupon]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<source><![CDATA[Hepatology]]></source>
<year>2009</year>
<volume>50</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>291-308</page-range></nlm-citation>
</ref>
<ref id="B10">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Natural history and management of primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Al-Harthy]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Kumagi]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<source><![CDATA[Hepat Med]]></source>
<year>2012</year>
<volume>4</volume>
<page-range>61-71</page-range></nlm-citation>
</ref>
<ref id="B11">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Ursodeoxycholic acid in the therapy for primary biliary cirrhosis effects on progression and prognosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Leuschner]]></surname>
<given-names><![CDATA[U]]></given-names>
</name>
<name>
<surname><![CDATA[Manns]]></surname>
<given-names><![CDATA[MP]]></given-names>
</name>
<name>
<surname><![CDATA[Eisebitt]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<source><![CDATA[Z Gastroenterol]]></source>
<year>2005</year>
<volume>43</volume>
<numero>9</numero>
<issue>9</issue>
<page-range>1051-9</page-range></nlm-citation>
</ref>
<ref id="B12">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Natural history of early primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Metcalf J]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Mitchison]]></surname>
<given-names><![CDATA[HC]]></given-names>
</name>
<name>
<surname><![CDATA[Palmer]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
</person-group>
<source><![CDATA[Lancet]]></source>
<year>1996</year>
<volume>348</volume>
<numero>9039</numero>
<issue>9039</issue>
<page-range>1399-402</page-range></nlm-citation>
</ref>
<ref id="B13">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Corpechot]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Abenavoli]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Rabahi]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<source><![CDATA[Hepatology]]></source>
<year>2008</year>
<volume>48</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>871-7</page-range></nlm-citation>
</ref>
<ref id="B14">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lammers]]></surname>
<given-names><![CDATA[WJ]]></given-names>
</name>
<name>
<surname><![CDATA[van-Buuren]]></surname>
<given-names><![CDATA[HR]]></given-names>
</name>
<name>
<surname><![CDATA[Hirschfield]]></surname>
<given-names><![CDATA[GM]]></given-names>
</name>
</person-group>
<source><![CDATA[Gastroenterology]]></source>
<year>2014</year>
<volume>147</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1338-49.e5</page-range></nlm-citation>
</ref>
<ref id="B15">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Poupon]]></surname>
<given-names><![CDATA[RE]]></given-names>
</name>
<name>
<surname><![CDATA[Lindor]]></surname>
<given-names><![CDATA[KD]]></given-names>
</name>
<name>
<surname><![CDATA[Cauch-Dudek]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<source><![CDATA[Gastroenterology]]></source>
<year>1997</year>
<volume>113</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>884-90</page-range></nlm-citation>
</ref>
<ref id="B16">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Corpechot]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Carrat]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Bahr]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<source><![CDATA[Gastroenterology]]></source>
<year>2005</year>
<volume>128</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>297-303</page-range></nlm-citation>
</ref>
<ref id="B17">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Utilization of the Mayo risk score in patients with primary biliary cirrhosis receiving ursodeoxycholic acid]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Angulo]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Lindor]]></surname>
<given-names><![CDATA[KD]]></given-names>
</name>
<name>
<surname><![CDATA[Therneau]]></surname>
<given-names><![CDATA[TM]]></given-names>
</name>
</person-group>
<source><![CDATA[Liver]]></source>
<year>1999</year>
<volume>19</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>115-21</page-range></nlm-citation>
</ref>
<ref id="B18">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Carbone]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Mells]]></surname>
<given-names><![CDATA[GF]]></given-names>
</name>
<name>
<surname><![CDATA[Pells]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<source><![CDATA[Gastroenterology]]></source>
<year>2013</year>
<volume>144</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>560-9</page-range></nlm-citation>
</ref>
<ref id="B19">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Baseline ductopenia and treatment response predict long-term histological progression in primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kumagi]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Guindi]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Fischer]]></surname>
<given-names><![CDATA[SE]]></given-names>
</name>
</person-group>
<source><![CDATA[Am J Gastroenterol]]></source>
<year>2010</year>
<volume>105</volume>
<numero>10</numero>
<issue>10</issue>
<page-range>2186-94</page-range></nlm-citation>
</ref>
<ref id="B20">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Optimizing biochemical markers as endpoints for clinical trials in primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Momah]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Silveira]]></surname>
<given-names><![CDATA[MG]]></given-names>
</name>
<name>
<surname><![CDATA[Jorgensen]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<source><![CDATA[Liver Int]]></source>
<year>2012</year>
<volume>32</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>790-5</page-range></nlm-citation>
</ref>
<ref id="B21">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Parés]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Caballería]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Rodés]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<source><![CDATA[Gastroenterology]]></source>
<year>2006</year>
<volume>130</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>715-20</page-range></nlm-citation>
</ref>
<ref id="B22">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Galiè]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Humbert]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Vachiery]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
</person-group>
<source><![CDATA[Eur Heart J]]></source>
<year>2016</year>
<volume>37</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>67-119</page-range></nlm-citation>
</ref>
<ref id="B23">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Living donor and deceased donor liver transplantation for autoimmune and cholestatic liver diseases - An analysis of the UNOS database]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kashyap]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Safadjou]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Chen]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<source><![CDATA[J Gastrointest Surg]]></source>
<year>2010</year>
<volume>14</volume>
<numero>9</numero>
<issue>9</issue>
<page-range>1362-9</page-range></nlm-citation>
</ref>
<ref id="B24">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[A placebo-controlled trial of obeticholic acid in primary biliary cholangitis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Nevens]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Andreone]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Mazzella]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<source><![CDATA[N Engl J Med]]></source>
<year>2016</year>
<volume>375</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>631-43</page-range></nlm-citation>
</ref>
<ref id="B25">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Primary biliary cirrhosis and liver transplantation]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Akamatsu]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Sugawara]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
</person-group>
<source><![CDATA[Intractable Rare Dis Res]]></source>
<year>2012</year>
<volume>1</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>66-80</page-range></nlm-citation>
</ref>
<ref id="B26">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Recent advances in the diagnosis and treatment of primary biliary cholangitis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Huang]]></surname>
<given-names><![CDATA[YQ]]></given-names>
</name>
</person-group>
<source><![CDATA[World J Hepatol]]></source>
<year>2016</year>
<volume>8</volume>
<numero>33</numero>
<issue>33</issue>
<page-range>1419-41</page-range></nlm-citation>
</ref>
<ref id="B27">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Extrahepatic manifestations of primary biliary cholangitis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Chalifoux]]></surname>
<given-names><![CDATA[SL]]></given-names>
</name>
<name>
<surname><![CDATA[Konyn]]></surname>
<given-names><![CDATA[PG]]></given-names>
</name>
<name>
<surname><![CDATA[Choi]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<source><![CDATA[Gut Liver]]></source>
<year>2017</year>
<volume>11</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>771-80</page-range></nlm-citation>
</ref>
<ref id="B28">
<nlm-citation citation-type="">
<collab>EMA (European Medicines Agency)</collab>
<source><![CDATA[Summary of Product Characteristics. Ocaliva]]></source>
<year>2016</year>
</nlm-citation>
</ref>
<ref id="B29">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[General knowledge and opinion of future health care and non-health care professionals on rare diseases]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ramalle-Gómara]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Ruiz]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Quiñones]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<source><![CDATA[J Eval Clin Pract]]></source>
<year>2015</year>
<volume>21</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>198-201</page-range></nlm-citation>
</ref>
<ref id="B30">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Incidence, prevalence and clinical course of primary biliary cirrhosis in a Spanish community]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Pla]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
<name>
<surname><![CDATA[Vergara]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Gil]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<source><![CDATA[Eur J Gastroenterol Hepatol]]></source>
<year>2007</year>
<volume>19</volume>
<numero>10</numero>
<issue>10</issue>
<page-range>859-64</page-range></nlm-citation>
</ref>
<ref id="B31">
<nlm-citation citation-type="">
<source><![CDATA[Registro Nacional de Enfermedades Colestásicas y Autoinmunes Hepáticas (ColHai) - AEEH]]></source>
<year>2017</year>
</nlm-citation>
</ref>
<ref id="B32">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Epidemiology of primary biliary cirrhosis in the south area of Granada]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Caballero Plasencia]]></surname>
<given-names><![CDATA[AM]]></given-names>
</name>
<name>
<surname><![CDATA[López Callejas]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Valenzuela Barranco]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<source><![CDATA[Med Clin (Barc)]]></source>
<year>1991</year>
<volume>96</volume>
<numero>13</numero>
<issue>13</issue>
<page-range>481-5</page-range></nlm-citation>
</ref>
<ref id="B33">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Epidemiology of primary biliary cirrhosis in southern Madrid]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Moreno Sánchez]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Cassinello Ogea]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[González Blanco]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
</person-group>
<source><![CDATA[Med Clin (Barc)]]></source>
<year>1990</year>
<volume>94</volume>
<numero>15</numero>
<issue>15</issue>
<page-range>564-9</page-range></nlm-citation>
</ref>
<ref id="B34">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Primary biliary cirrhosis in Navarra]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Borda]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Huarte]]></surname>
<given-names><![CDATA[MP]]></given-names>
</name>
<name>
<surname><![CDATA[Zozaya]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
</person-group>
<source><![CDATA[An Med Interna]]></source>
<year>1989</year>
<volume>6</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>63-6</page-range></nlm-citation>
</ref>
<ref id="B35">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Epidemiology of primary biliary cirrhosis in Cataluña]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Parés]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bruguera]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Rodés]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<source><![CDATA[Med Clin (Barc)]]></source>
<year>1984</year>
<volume>82</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>237-41</page-range></nlm-citation>
</ref>
<ref id="B36">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Epidemiology of primary biliary cirrhosis in Asturias]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rodrigo Sáez]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Valerdiz Casasola]]></surname>
<given-names><![CDATA[CS]]></given-names>
</name>
<name>
<surname><![CDATA[Rodríguez García]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<source><![CDATA[Med Clin (Barc)]]></source>
<year>1987</year>
<volume>88</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>486-9</page-range></nlm-citation>
</ref>
<ref id="B37">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[The Delphi technique in health sciences education research]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[De Villiers]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[De Villiers]]></surname>
<given-names><![CDATA[PJT]]></given-names>
</name>
<name>
<surname><![CDATA[Kent]]></surname>
<given-names><![CDATA[AP]]></given-names>
</name>
</person-group>
<source><![CDATA[Med Teach]]></source>
<year>2005</year>
<volume>27</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>639-43</page-range></nlm-citation>
</ref>
<ref id="B38">
<nlm-citation citation-type="">
<source><![CDATA[Albi España. Asociación para la Lucha contra las Enfermedades Biliares Inflamatorias]]></source>
<year>2017</year>
</nlm-citation>
</ref>
<ref id="B39">
<nlm-citation citation-type="">
<source><![CDATA[AEEH. Asociación Española para el Estudio del Hígado]]></source>
<year>2017</year>
</nlm-citation>
</ref>
<ref id="B40">
<nlm-citation citation-type="">
<source><![CDATA[Orphanet. Orphan Drugs in Europe]]></source>
<year>2017</year>
</nlm-citation>
</ref>
<ref id="B41">
<nlm-citation citation-type="">
<source><![CDATA[INE. Instituto Nacional de Estadística]]></source>
<year>2017</year>
</nlm-citation>
</ref>
<ref id="B42">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Rising incidence and prevalence of primary biliary cirrhosis a large population-based study]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Boonstra]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Kunst]]></surname>
<given-names><![CDATA[AE]]></given-names>
</name>
<name>
<surname><![CDATA[Stadhouders]]></surname>
<given-names><![CDATA[PH]]></given-names>
</name>
</person-group>
<source><![CDATA[Liver Int]]></source>
<year>2014</year>
<volume>34</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>e31-8</page-range></nlm-citation>
</ref>
<ref id="B43">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[The limitations and hidden gems of the epidemiology of primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Podda]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Selmi]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Lleo]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<source><![CDATA[J Autoimmun]]></source>
<year>2013</year>
<volume>46</volume>
<page-range>81-7</page-range></nlm-citation>
</ref>
<ref id="B44">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Prevalence and incidence of primary biliary cirrhosis are increasing in Finland]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rautiainen]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Salomaa]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Niemelä]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<source><![CDATA[Scand J Gastroenterol]]></source>
<year>2007</year>
<volume>42</volume>
<numero>11</numero>
<issue>11</issue>
<page-range>1347-53</page-range></nlm-citation>
</ref>
<ref id="B45">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Primary biliary cholangitis in China]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Gershwin]]></surname>
<given-names><![CDATA[ME]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[FS]]></given-names>
</name>
</person-group>
<source><![CDATA[Curr Opin Gastroenterol]]></source>
<year>2016</year>
<volume>32</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>1</page-range></nlm-citation>
</ref>
<ref id="B46">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[Epidemiology and natural history of primary biliary cirrhosis in a Canadian health region a population-based study]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Myers]]></surname>
<given-names><![CDATA[RP]]></given-names>
</name>
<name>
<surname><![CDATA[Shaheen]]></surname>
<given-names><![CDATA[AAM]]></given-names>
</name>
<name>
<surname><![CDATA[Fong]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<source><![CDATA[Hepatology]]></source>
<year>2009</year>
<volume>50</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>1884-92</page-range></nlm-citation>
</ref>
<ref id="B47">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[An integrated care pathway improves quality of life in primary biliary cirrhosis]]></article-title>
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jones]]></surname>
<given-names><![CDATA[DEJ]]></given-names>
</name>
<name>
<surname><![CDATA[Sutcliffe]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Pairman]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<source><![CDATA[QJM]]></source>
<year>2008</year>
<volume>101</volume>
<numero>7</numero>
<issue>7</issue>
<page-range>535-43</page-range></nlm-citation>
</ref>
<ref id="B48">
<nlm-citation citation-type="journal">
<article-title xml:lang=""><![CDATA[EASL Clinical Practice Guidelines: Management of cholestatic liver diseases]]></article-title>
<collab>European Association for the Study of the Liver</collab>
<source><![CDATA[J Hepatol]]></source>
<year>2009</year>
<volume>51</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>237-67</page-range></nlm-citation>
</ref>
<ref id="B49">
<nlm-citation citation-type="">
<collab>Comisión Interministerial de Precios de los Medicamentos</collab>
<source><![CDATA[Nota informativa de la reunión de la comisión interministerial de precios de los medicamentos. Sesión 177 de 6 de noviembre de 2017]]></source>
<year>2018</year>
</nlm-citation>
</ref>
<ref id="B50">
<nlm-citation citation-type="">
<collab>AEMPS</collab>
<source><![CDATA[Ficha técnica Ocaliva 5 mg comprimidos recubiertos con película]]></source>
<year>2018</year>
</nlm-citation>
</ref>
</ref-list>
</back>
</article>
