<?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>2393-6797</journal-id>
<journal-title><![CDATA[Revista Uruguaya de Medicina Interna ]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. Urug. Med. Int.]]></abbrev-journal-title>
<issn>2393-6797</issn>
<publisher>
<publisher-name><![CDATA[Sociedad de Medicina Interna del Uruguay]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2393-67972018000100015</article-id>
<article-id pub-id-type="doi">10.26445/rmu.3.1.2</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Esclerosis sistémica: forma de presentación y manejo terapéutico: experiencia de un grupo de trabajo en Enfermedades Autoinmunes Sistémicas.]]></article-title>
<article-title xml:lang="en"><![CDATA[Systemic sclerosis: clinical presentation and therapeutic management: experience from a systemic disease network.]]></article-title>
<article-title xml:lang="pt"><![CDATA[Esclerose sistêmica: forma de apresentação e manejo terapéutico: experiência de um grupo de trabalho em doenças sistêmicas autoimunes.]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Graña]]></surname>
<given-names><![CDATA[Diego]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Vargas]]></surname>
<given-names><![CDATA[Andrea]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Bérez]]></surname>
<given-names><![CDATA[Adriana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Goñi]]></surname>
<given-names><![CDATA[Mabel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Danza]]></surname>
<given-names><![CDATA[Alvaro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad de la República Grupo de Trabajo en Enfermedades Autoinmunes Sistémicas ]]></institution>
<addr-line><![CDATA[Montevideo ]]></addr-line>
<country>Uruguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>04</month>
<year>2018</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>04</month>
<year>2018</year>
</pub-date>
<volume>3</volume>
<numero>1</numero>
<fpage>15</fpage>
<lpage>22</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S2393-67972018000100015&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S2393-67972018000100015&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S2393-67972018000100015&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN:  Introducción:  La esclerosis sistémica (ES) es una enfermedad crónica y heterogénea caracterizada por fibrosis de la piel y órganos internos, vasculopatía de pequeños vasos, asociada a autoanticuerpos. El objetivo del estudio fue conocer la frecuencia, forma de presentación y tratamientos practicados en nuestro medio (Uruguay).  Material y Método: Se realizó un estudio retrospectivo de pacientes con diagnóstico de ES (según ACR/EULAR 2013), asistidos en nuestro Grupo Trabajo en Enfermedades Autoinmunes Sistémicas. Se analizaron las siguientes variables: edad, sexo, perfil clínico, compromiso parenquimatoso predominante, autoanticuerpos, capilaroscopía, tratamiento inmunosupresor recibido en los últimos 6 meses, motivo deindicación.  Resultados: Se incluyeron 49 casos de ES. La mediana de edad fue 63 (25-85) años, 44 (90%) de sexo femenino. Perfil clínico: 5 (10%) ES sin esclerodermia, 19 (39%) ES difusa, 25 (51%) ES limitada. El parénquima predominantemente afectado fue: cutáneo en 42 (85,7%) casos, Raynaud 41 (83,7%), articular 27 (55,1%), tubo digestivo 24 (49%), enfermedad pulmonar intersticial (EPI) 16 (32,7%), hipertensión pulmonar 7 (14,3%), renal 3 (6,1%) y otros 2 (4,1%). Recibieron tratamiento inmunosupresor 17 (34,6%), siendo el motivo de inmunosupresión en 8 (16,3%) casos el compromiso respiratorio, en 5 (10,2%) el cutáneo y en 4 (8,2%) el articular. Este tratamiento se empleó más frecuentemente en la ES difusa (10 casos, 20,4%).  Conclusiones: En nuestra serie predomina la ES limitada, el sexo femenino, compromiso cutáneo, fenómeno de Raynaud y manifestaciones articulares. Un tercio de los pacientes presentaron EPI. El uso de inmunosupresores predominó en pacientes con compromiso respiratorio y en la forma difusa.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT:  Introduction: Systemic sclerosis (ES) is a chronic and heterogeneous disease characterized by fibrosis of the skin and internal organs, small vessel vasculopathy, associated with autoantibodies. The objective of the study was to know the frequency, form of presentation and treatments practiced in our environment (Uruguay).  Material and method:  A retrospective study of patients diagnosed with ES (according to ACR / EULAR 2013), assisted in our Working Group on Systemic Autoimmune Diseases, was conducted. The following variables were analyzed: age, sex, clinical profile, predominant parenchymal commitment, autoantibodies, capillaroscopy, immunosuppressive treatment received in the last 6 months, reason for indication. Results. 49 cases of ES were included. The median age was 63 (25-85) years, 44 (90%) female. Clinical profile: 5 (10%) ES without scleroderma, 19 (39%) ES diffuse, 25 (51%) IS limited. The predominantly affected parenchyma was: cutaneous in 42 (85.7%) cases, Raynaud 41 (83.7%), articular 27 (55.1%), gastrointestinal tract 24 (49%), interstitial lung disease (EPI) 16 (32.7%), pulmonary hypertension 7 (14.3%), renal 3 (6.1%) and other 2 (4.1%). They received immunosuppressive treatment 17 (34.6%), being the reason for immunosuppression in 8 (16.3%) cases the respiratory compromise, in 5 (10.2%) the cutaneous and in 4 (8.2%) the joint . This treatment was used more frequently in diffuse ES (10 cases, 20.4%).  Conclusions: In our series, limited SS, female sex, skin involvement, Raynaud's phenomenon and joint manifestations predominate. One third of patients presented with PID. The use of immunosuppressants predominated in patients with respiratory compromise and in the diffuse form.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo:  Introdução: A esclerose sistêmica (ES) é uma doença crônica e heterogênea caracterizada por fibrose da pele e órgãos internos, vasculopatia de pequeno vaso, associada a autoanticorpos. O objetivo do estudo foi conhecer a freqüência, a forma de apresentação e os tratamentos praticados no nosso meio ambiente (Uruguai).  Material e Método: Realizou-se um estudo retrospectivo de pacientes diagnosticados com ES (de acordo com ACR / EULAR 2013), assistido em nosso Grupo de Trabalho sobre Doenças Autoimunes Sistêmicas. Foram analisadas as seguintes variáveis: idade, sexo, perfil clínico, comprometimento parenquimatoso predominante, autoanticorpos, capilaroscopia, tratamento imunossupressor recebido nos últimos 6 meses, motivo de indicação. Resultados. Foram incluídos 49 casos de ES. A idade mediana foi de 63 (25-85) anos, 44 (90%) do sexo feminino. Perfil clínico: 5 (10%) ES sem esclerodermia, 19 (39%) ES difuso, 25 (51%) IS limitado. O parênquima predominantemente afetado foi: cutâneo em 42 (85,7%) casos, Raynaud 41 (83,7%), articular 27 (55,1%), trato gastrointestinal 24 (49%), doença pulmonar intersticial (EPI) 16 (32,7%), hipertensão pulmonar 7 (14,3%), renal 3 (6,1%) e outros 2 (4,1%). Eles receberam tratamento imunossupressor 17 (34,6%), sendo motivo de imunossupressão em 8 (16,3%) casos o comprometimento respiratório, em 5 (10,2%) cutâneo e em 4 (8,2%) a articulação . Este tratamento foi utilizado mais frequentemente em ES difusa (10 casos, 20,4%).  Conclusões:  Em nossa série, SSs limitados, sexo feminino, envolvimento da pele, fenômeno de Raynaud e manifestações articulares predominam. Um terço dos pacientes apresentou PID. O uso de imunossupressores predominou em pacientes com comprometimento respiratório e na forma difusa.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[esclerosis sistémica]]></kwd>
<kwd lng="es"><![CDATA[autoinmunidad]]></kwd>
<kwd lng="es"><![CDATA[terapéutica]]></kwd>
<kwd lng="en"><![CDATA[systemic sclerosis]]></kwd>
<kwd lng="en"><![CDATA[autoimmunity]]></kwd>
<kwd lng="en"><![CDATA[therapeutics]]></kwd>
<kwd lng="pt"><![CDATA[esclerose sistêmica]]></kwd>
<kwd lng="pt"><![CDATA[auto-imunidade]]></kwd>
<kwd lng="pt"><![CDATA[terapêutica]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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