<?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>0121-8123</journal-id>
<journal-title><![CDATA[Revista Colombiana de Reumatología]]></journal-title>
<abbrev-journal-title><![CDATA[Rev.Colomb.Reumatol.]]></abbrev-journal-title>
<issn>0121-8123</issn>
<publisher>
<publisher-name><![CDATA[Asociación Colombiana de Reumatología]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0121-81232020000500135</article-id>
<article-id pub-id-type="doi">10.1016/j.rcreu.2020.05.026</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Pharmacological treatment of systemic sclerosis-associated pulmonary hypertension: A systematic literature review]]></article-title>
<article-title xml:lang="es"><![CDATA[Tratamiento farmacológico de la hipertensión pulmonar asociada a la esclerosis sistémica: revisión sistemática de la literatura]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cajas Santana]]></surname>
<given-names><![CDATA[Luis Javier]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Florez Suarez]]></surname>
<given-names><![CDATA[Jorge Bruce]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Méndez Toro]]></surname>
<given-names><![CDATA[Arnold]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Quintana López]]></surname>
<given-names><![CDATA[Gerardo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Nacional de Colombia  ]]></institution>
<addr-line><![CDATA[Bogotá, D.C. ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Fundación Santa Fe de Bogotá  ]]></institution>
<addr-line><![CDATA[Bogotá, D.C. ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidad Nacional de Colombia  ]]></institution>
<addr-line><![CDATA[Bogotá, D.C. ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Institute of Rheumatology  ]]></institution>
<addr-line><![CDATA[Bogotá, D.C. ]]></addr-line>
<country>Colombia</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2020</year>
</pub-date>
<volume>27</volume>
<fpage>135</fpage>
<lpage>145</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0121-81232020000500135&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0121-81232020000500135&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0121-81232020000500135&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Background: Systemic sclerosis is an autoimmune disease that could significantly affect internal organs, increasing the mortality associated with the disorder. Pulmonary hypertension is one of the conditions that increase the morbidity and mortality of the disease. The aim of the study was to develop a systematic literature review about the different pharmacological treatments for systemic sclerosis-associated pulmonary hypertension.  Materials and methods: A systematic literature review was made on Cochrane, PubMed and EMBASE. Randomized controlled clinical trials, controlled clinical trials, cohort and case-control studies using any of the available treatments such as prostacyclin analogs, endothelin receptor antagonists, phosphodiesterase inhibitors or soluble guanylate cyclase stimulants were included. The methodological quality of the studies was assessed using Cochrane methodology for clinical trials, and the SIGN tool was applied for cohort and case-control studies.  Results:  Initially, 870 studies were found, and 20 studies were finally included in the systematic review after applying inclusion and exclusion criteria. Most studies included Bosentan in the treatment strategies (N = 13). Also, most studies took data from the most important clinical trials for pharmacological treatment of pulmonary hypertension of several etiologies. Some studies using Bosentan did not give information about data regarding some outcomes. Half of the selected studies were clinical trials, of which two were randomized. The quality of these studies was good. The other half were observational studies, with moderate quality of evidence. In general, it was found that approved medications improve the 6-minute walk distance, WHO functional class, NYHA functional classification, or Borg dyspnea score, depending on the given dose in several studies, and also compound outcomes that include hospitalizations, clinical worsening, death, etc. Regarding mortality, there were significant differences in survival rates when comparing approved medications vs. placebo, being combined therapies superior to monotherapy. Measurements of hemodynamic variables such as cardiac index, pulmonary resistances, and pulmonary pressures were improved. Also, NT-proBNP levels were reduced, with some exceptions.  Conclusions: In most studies with good quality of evidence, it was found an improvement in clinical and non-clinical outcomes, favoring the use of approved medications for the treatment of systemic sclerosis-associated pulmonary hypertension.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Antecedentes:  La esclerosis sistémica es una enfermedad autoinmune que puede afectar significativamente los órganos internos, aumentando la mortalidad asociada con dicho trastorno. La hipertensión pulmonar es una de las afecciones que aumentan la morbimortalidad de la enfermedad. El objetivo del estudio fue desarrollar una revisión sistemática de la literatura sobre los diferentes tratamientos farmacológicos para la hipertensión pulmonar asociada a la esclerosis sistémica.  Materiales y métodos:  Se hizo una revisión sistemática de la literatura en Cochrane, PubMed y EMBASE. Se incluyeron ensayos clínicos aleatorizados controlados, ensayos clínicos controlados, así como estudios de cohortes y de casos y controles utilizando alguno de los tratamientos disponibles, tales como los análogos de la prostaciclina, los antagonistas de los receptores de endotelina, los inhibidores de la fosfodiesterasa o los estimulantes de la guanilato ciclasa soluble. Se evaluó la calidad metodológica de los estudios utilizando la metodología Cochrane para los ensayos clínicos, y se aplicó la herramienta SIGN para los estudios de cohortes y de casos y controles.  Resultados:  Inicialmente se encontraron 870 estudios, de los cuales finalmente se incluyeron 20 en la revisión sistemática, después de aplicar los criterios de inclusión y exclusión. La mayoría de los estudios incluyeron el bosentán en las estrategias de tratamiento (n = 13). Además, la mayoría de los estudios tomaron datos de los ensayos clínicos más importantes sobre el tratamiento farmacológico de la hipertensión pulmonar de diversas etiologías. Algunos estudios que utilizaron el bosentán no suministraron información sobre datos relacionados con algunos resultados. La mitad de los estudios seleccionados fueron ensayos clínicos, de los cuales dos fueron aleatorizados. La calidad de estos estudios fue buena. La otra mitad incluyó estudios observacionales, con evidencia de calidad moderada. En general, se encontró que los medicamentos aprobados mejoran la distancia en la caminata de seis minutos, la clase funcional de la OMS, la clasificación funcional de la NYHA, o el puntaje de disnea en la escala de Borg, dependiendo de la dosis administrada en los diferentes estudios, y también los desenlaces compuestos que incluyen hospitalizaciones, empeoramiento clínico, muerte, etc. En cuanto a la mortalidad, se encontraron diferencias significativas en las tasas de supervivencia al comparar los medicamentos aprobados versus placebo, siendo las terapias combinadas superiores a la monoterapia. Las mediciones de variables hemodinámicas tales como el índice cardíaco, las resistencias pulmonares y las presiones pulmonares mejoraron. Además, los niveles de NT-pro BNP se redujeron, con algunas excepciones.  Conclusiones:  En la mayoría de los estudios con buena calidad de evidencia, se encontró una mejoría en los desenlaces clínicos y no clínicos, que favorece el uso de los medicamentos aprobados para el tratamiento de la hipertensión pulmonar asociada a la esclerosis sistémica.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Systemic sclerosis]]></kwd>
<kwd lng="en"><![CDATA[Scleroderma]]></kwd>
<kwd lng="en"><![CDATA[Pulmonary hypertension]]></kwd>
<kwd lng="en"><![CDATA[Treatment]]></kwd>
<kwd lng="es"><![CDATA[Esclerosis sistémica Escleroderma]]></kwd>
<kwd lng="es"><![CDATA[Hipertensión pulmonar]]></kwd>
<kwd lng="es"><![CDATA[Tratamiento]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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