<?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>S0121-81232024000500082</article-id>
<article-id pub-id-type="doi">10.1016/j.rcreu.2023.09.007</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Progressive pulmonary fibrosis associated autoimmune diseases: Systematic review of the literature]]></article-title>
<article-title xml:lang="es"><![CDATA[Fibrosis pulmonar progresiva asociada a enfermedad autoinmune: 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"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gil Calderón]]></surname>
<given-names><![CDATA[Diana Rocío]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rubio Rivera]]></surname>
<given-names><![CDATA[Manuela]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Yate]]></surname>
<given-names><![CDATA[José Alex]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Galindo]]></surname>
<given-names><![CDATA[Javier Leonardo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Colombia National University Hospital Department of Rheumatology ]]></institution>
<addr-line><![CDATA[Bogotá ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,National University of Colombia Faculty of Medicine Department of Rheumatology]]></institution>
<addr-line><![CDATA[Bogotá ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Mederi Major University Hospital Department of Rheumatology ]]></institution>
<addr-line><![CDATA[Bogotá ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Artmedica SAS Service of Rheumatology ]]></institution>
<addr-line><![CDATA[Medellín ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,Del Valle University Hospital Department of Rheumatology ]]></institution>
<addr-line><![CDATA[Cali ]]></addr-line>
<country>Colombia</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2024</year>
</pub-date>
<volume>31</volume>
<fpage>82</fpage>
<lpage>93</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0121-81232024000500082&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0121-81232024000500082&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0121-81232024000500082&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  Connective tissue disease-related interstitial lung disease (CTD-ILD) accounts for 30% of all cases of ILD. Some patients progress and develop progressive pulmonary fibrosis, which has a prognosis comparable to interstitial pulmonary fibrosis. In this study, relevant evidence about epidemiology, risk factors, biomarkers, and treatment are reviewed.  Materials and methods:  A systematic review of the literature was carried out. Original observational and descriptive articles were included. Articles not providing information about the CTD diagnosis were excluded. PUBMED, EMBASE, SCOPUS, and LILACS were all searched. The total number of articles obtained was 528; data were extracted from 61 original articles.  Results:  On average, ILD in these patients progressed by 33.7% over time. Patients with progressive pulmonary fibrosis had a similar 3.7-year median survival as those with idiopathic pulmonary fibrosis. Mortality was markedly increased (hazards ratio 3.29; 95% CI 2.76-3.82). A progressive course was seen in 34% of scleroderma (SSc) related ILD cases. Six interferon-induced proteins and a proteomic profile of 12 biomarkers were used to predict progression and response to treatment. The INBUILD and SENSCIS studies that assessed the effectiveness of nintedanib effectiveness revealed a reduced decrease in forced vital capacity. Progression in rheumatoid arthritis (RA) related ILD ranges from 38% to 50%. Control of RA disease activity and use of antifibrotics benefit the lungs.  Conclusion:  A significant proportion of patients with CTD-ILD have progressive lung disease, with the corresponding adverse mortality effects. The majority of the data regarding CTD-ILD are from cohorts of patients with RA-ILD and SSc-ILD, in which antifibrotics and concurrent immunosuppressive treatments have been effective. There is not enough information available on other autoimmune disorders to draw any firm conclusions regarding progression rates or treatment effects.]]></p></abstract>
<abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  La enfermedad pulmonar intersticial (EPI) relacionada con la enfermedad del tejido conectivo (ETC-EPI) representa el 30% de todos los casos de EPI. Algunos pacientes progresan y desarrollan fibrosis pulmonar progresiva, que tiene un pronóstico comparable al de la fibrosis pulmonar intersticial. En este estudio se revisa la evidencia relevante sobre la epidemiología, los factores de riesgo, los biomarcadores y el tratamiento.  Materiales y métodos:  Se realizó una revisión sistemática de la literatura. Se incluyeron artículos originales observacionales y descriptivos. Se excluyeron los artículos que no proporcionaban información sobre el diagnóstico de ETC. Se realizaron búsquedas en Pubmed, Embase, Scopus y LILACS. El número total de artículos obtenidos fue de 528; los datos se extrajeron de 61 artículos originales.  Resultados:  En promedio, la EPI en estos pacientes progresó un 33,7% con el tiempo. Los pacientes con fibrosis pulmonar progresiva tuvieron una mediana de supervivencia de 3,7 anos, similar a la de aquellos con fibrosis pulmonar idiopática. La mortalidad aumentó notablemente (índice de riesgos: 3,29; IC 95%: 2,76-3,82). Se observó un curso progresivo en el 34% de los casos de EPI relacionados con la esclerodermia (ES). Se utilizaron 6 proteínas inducidas por interferón y un perfil proteómico de 12 biomarcadores para predecir progresión y respuesta al tratamiento. Los estudios INBUILD y SENSCIS que evaluaron la eficacia de nintedanib revelaron una disminución reducida de la capacidad vital forzada. La progresión de la EPI relacionada con la artritis reumatoide (AR) oscila entre el 38 y el 50%. El control de la actividad de la enfermedad y el uso de antifibróticos benefician a los pulmones.  Conclusión:  Una proporción significativa de pacientes con ETC-EPI tienen enfermedad pulmonar progresiva, con los correspondientes efectos adversos de mortalidad. La mayoría de los datos sobre ETC-EPI provienen de cohortes de pacientes con AR-EPI y SSc-EPI, en los cuales los antifibróticos y los tratamientos inmunosupresores concurrentes han sido efectivos. No hay suficiente información disponible sobre otros trastornos autoinmunes para llegar a conclusiones firmes sobre las tasas de progresión o los efectos del tratamiento.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Progressive fibrosing interstitial lung disease]]></kwd>
<kwd lng="en"><![CDATA[Progressive fibrosing]]></kwd>
<kwd lng="en"><![CDATA[ILD PF-ILD]]></kwd>
<kwd lng="es"><![CDATA[Enfermedad pulmonar intersticial fibrosante progresiva]]></kwd>
<kwd lng="es"><![CDATA[EPI fibrosante progresiva]]></kwd>
<kwd lng="es"><![CDATA[PF-EPI]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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