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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Idiopathic inflammatory myopathies &#40;IIM&#41;&#44; including dermatomyositis &#40;DM&#41;&#44; are a heterogeneous group of systemic autoimmune diseases whose main clinical feature is predominantly proximal muscle weakness&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> They are rare diseases&#44; with an estimated annual incidence of 6 cases&#47;10<span class="elsevierStyleSup">5</span> individuals<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> and may be associated with other autoimmune diseases such as lupus or Sjogren&#39;s syndrome&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Another autoimmune disease that causes muscle weakness&#44; myasthenia gravis &#40;MG&#41;&#44; can also be associated with the IIM&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3&#8211;9</span></a> MG is rare&#44; with an estimated prevalence of 140 cases&#47;10<span class="elsevierStyleSup">6</span> inhabitants&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> Its pathogenesis derives from the compromise resulting from the binding of autoantibodies to proteins involved in the neuromuscular transmission signaling&#46; Recognizing the association between the two entities is important due to its therapeutic implications&#46; We present a case of DM in a patient previously diagnosed with MG&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Case</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 69 year-old-male Caucasian&#44; diagnosed two years prior with MG&#44; positive to anti-AChR antibodies and treated with pyridostigmine &#40;60<span class="elsevierStyleHsp" style=""></span>mg&#47;8<span class="elsevierStyleHsp" style=""></span>h&#41;&#46; He was referred to our center due to the appearance of weakness which appeared a month before&#44; manifested by difficulty dressing or getting out of bed&#44; shoulder joint pain and elevated creatine kinase &#40;CK&#41;&#46; He had presented diffuse facial erythema for 6 months&#46; The physical examination showed cuticular thickening and bleeding&#44; Gottron&#39;s papules &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and facial erythema and on the neckline &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41; He had proximal muscle weakness in the upper and lower limbs &#40;4&#47;5&#41;&#46; The rest of the examination was normal&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The laboratory exams highlighted CK 1254<span class="elsevierStyleHsp" style=""></span>U&#47;l&#46; Antinuclear antibodies were positive &#40;&#62;1&#47;160&#44; with a granular pattern&#44; and anti-ENA and anti-DNA were negative&#41; as well as the anti-Mi2&#46; Nailfold capillaroscopy showed a pattern suggestive of DM &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Electromyography showed a myopathic pattern with proximal predominance in the upper limbs&#46; A muscle biopsy was not considered necessary&#46; Given the high sensitivity of the test for occult neoplasia&#44; a PET-CT was performed and was normal&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">With the diagnosis of DM&#44; prednisone &#40;1<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41; was started&#46; After three weeks&#44; significant improvement of skin lesions was observed&#44; but muscle weakness persisted&#44; with added dysphagia&#46; CK levels increased to 3800<span class="elsevierStyleHsp" style=""></span>&#956;&#47;l&#46; Weekly methotrexate &#40;h 20<span class="elsevierStyleHsp" style=""></span>mg&#47;week SC&#41; and a course of high-dose intravenous immunoglobulins was added to treatment&#46; The patient showed improvement in muscle strength and swallowing&#44; as well as disappearance of Gottron&#39;s papules&#44; with gradual decrease in CK&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">The association of MG to MII is very rare&#44; with 26 cases<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3&#8211;9</span></a> having been communicated until the present&#46; It is important to know of this association because it has therapeutic implications&#46; Unlike patients with IIM&#44; MG presents with fluctuating weakness that worsens with activity and during the course of the day&#46; In addition&#44; a real muscle fatigability in IIM is not characteristic&#44; while it is a typical clinical characteristic of MG&#46; In most patients with MG&#44; the eye muscles are initially affected&#44; causing intermittent diplopia and ptosis&#44; symptoms that are not observed in the IIM&#46; Proximal muscle weakness is a common symptom of MG and in DM&#44; appearing with bulbar symptoms such as dysphagia or dysarthria&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Treatment of MG includes anticholinesterase although not all patients respond&#46; As in IIM&#44; glucocorticoids&#44; immunosuppressants and immunomodulators&#8211;intravenous immunoglobulin&#8211;are frequently used in MG&#46; The usual pattern of steroid use in DM is high prednisone doses &#40;1<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleMonospace">&#47;</span>kg&#47;day&#41; with subsequent gradual tapering&#46; However&#44; in MG it is recommended to start with a low dose and gradually increase due to risk of exacerbation of weakness&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In summary&#44; MG may precede or complicate the course of IIM&#46; Knowing this association is important because it requires different handling and treatment&#44; with a gradual intensification of therapy with glucocorticoids&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Ethical Responsibilities</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Protection of people and animals</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare this research did not perform experiments on humans or animals&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Data confidentiality</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their workplace regarding the publication of patient data&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Right to privacy and informed consent</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors have obtained the informed consent of patients and&#47;or subjects referred to in the article&#46; This document is in the possession of the corresponding author&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflict of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors have no conflict of interest to state&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The association of dermatomyositis with myasthenia gravis &#40;MG&#41; is uncommon&#44; having been reported so far in only 26 cases&#46; We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne&#46; The patient developed acute proximal weakness&#44; shoulder pain and elevated creatine-kinase &#40;CK&#41;&#46; He also developed generalized facial erythema and Gottron&#39;s papules&#46; Laboratory tests showed positive antinuclear and anti-Mi2 antibodies&#46; Further analysis confirmed CK levels above 1000<span class="elsevierStyleHsp" style=""></span>U&#47;l&#46; The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discussed&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Sang&#252;esa G&#243;mez C&#44; Flores Robles BJ&#44; M&#233;ndez Perles C&#44; Barbadillo C&#44; Godoy H&#44; Andr&#233;u JL&#46; Dermatomiositis y miastenia gravis&#58; una asociaci&#243;n infrecuente con implicaciones terap&#233;uticas&#46; Reumatol Clin&#46; 2015&#59;11&#58;244&#8211;246&#46;</p>"
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                            0 => "C&#46; Vasilescu"
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                            2 => "P&#46;E&#46; Vos"
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Case Report
Dermatomyositis and Myasthenia Gravis: An Uncommon Association With Therapeutic Implications
Dermatomiositis y miastenia gravis: una asociación infrecuente con implicaciones terapéuticas
Clara Sangüesa Gómez, Bryan Josué Flores Robles, Clara Méndez Perles, Carmen Barbadillo, Hildegarda Godoy, José Luis Andréu
Corresponding author
jlandreu@arrakis.es

Corresponding author.
Servicio de Reumatología, Hospital Puerta de Hierro-Majadahonda, Majadahonda, Madrid, Spain
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myasthenia gravis &#40;MG&#41;&#44; can also be associated with the IIM&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3&#8211;9</span></a> MG is rare&#44; with an estimated prevalence of 140 cases&#47;10<span class="elsevierStyleSup">6</span> inhabitants&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> Its pathogenesis derives from the compromise resulting from the binding of autoantibodies to proteins involved in the neuromuscular transmission signaling&#46; Recognizing the association between the two entities is important due to its therapeutic implications&#46; We present a case of DM in a patient previously diagnosed with MG&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Case</span><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 69 year-old-male Caucasian&#44; diagnosed two years prior with MG&#44; positive to anti-AChR antibodies and treated with pyridostigmine &#40;60<span class="elsevierStyleHsp" style=""></span>mg&#47;8<span class="elsevierStyleHsp" style=""></span>h&#41;&#46; He was referred to our center due to the appearance of weakness which appeared a month before&#44; manifested by difficulty dressing or getting out of bed&#44; shoulder joint pain and elevated creatine kinase &#40;CK&#41;&#46; He had presented diffuse facial erythema for 6 months&#46; The physical examination showed cuticular thickening and bleeding&#44; Gottron&#39;s papules &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and facial erythema and on the neckline &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41; He had proximal muscle weakness in the upper and lower limbs &#40;4&#47;5&#41;&#46; The rest of the examination was normal&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The laboratory exams highlighted CK 1254<span class="elsevierStyleHsp" style=""></span>U&#47;l&#46; Antinuclear antibodies were positive &#40;&#62;1&#47;160&#44; with a granular pattern&#44; and anti-ENA and anti-DNA were negative&#41; as well as the anti-Mi2&#46; Nailfold capillaroscopy showed a pattern suggestive of DM &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Electromyography showed a myopathic pattern with proximal predominance in the upper limbs&#46; A muscle biopsy was not considered necessary&#46; Given the high sensitivity of the test for occult neoplasia&#44; a PET-CT was performed and was normal&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">With the diagnosis of DM&#44; prednisone &#40;1<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41; was started&#46; After three weeks&#44; significant improvement of skin lesions was observed&#44; but muscle weakness persisted&#44; with added dysphagia&#46; CK levels increased to 3800<span class="elsevierStyleHsp" style=""></span>&#956;&#47;l&#46; Weekly methotrexate &#40;h 20<span class="elsevierStyleHsp" style=""></span>mg&#47;week SC&#41; and a course of high-dose intravenous immunoglobulins was added to treatment&#46; The patient showed improvement in muscle strength and swallowing&#44; as well as disappearance of Gottron&#39;s papules&#44; with gradual decrease in CK&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">The association of MG to MII is very rare&#44; with 26 cases<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3&#8211;9</span></a> having been communicated until the present&#46; It is important to know of this association because it has therapeutic implications&#46; Unlike patients with IIM&#44; MG presents with fluctuating weakness that worsens with activity and during the course of the day&#46; In addition&#44; a real muscle fatigability in IIM is not characteristic&#44; while it is a typical clinical characteristic of MG&#46; In most patients with MG&#44; the eye muscles are initially affected&#44; causing intermittent diplopia and ptosis&#44; symptoms that are not observed in the IIM&#46; Proximal muscle weakness is a common symptom of MG and in DM&#44; appearing with bulbar symptoms such as dysphagia or dysarthria&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Treatment of MG includes anticholinesterase although not all patients respond&#46; As in IIM&#44; glucocorticoids&#44; immunosuppressants and immunomodulators&#8211;intravenous immunoglobulin&#8211;are frequently used in MG&#46; The usual pattern of steroid use in DM is high prednisone doses &#40;1<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleMonospace">&#47;</span>kg&#47;day&#41; with subsequent gradual tapering&#46; However&#44; in MG it is recommended to start with a low dose and gradually increase due to risk of exacerbation of weakness&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In summary&#44; MG may precede or complicate the course of IIM&#46; Knowing this association is important because it requires different handling and treatment&#44; with a gradual intensification of therapy with glucocorticoids&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Ethical Responsibilities</span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Protection of people and animals</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare this research did not perform experiments on humans or animals&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Data confidentiality</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their workplace regarding the publication of patient data&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Right to privacy and informed consent</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors have obtained the informed consent of patients and&#47;or subjects referred to in the article&#46; This document is in the possession of the corresponding author&#46;</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflict of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors have no conflict of interest to state&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The association of dermatomyositis with myasthenia gravis &#40;MG&#41; is uncommon&#44; having been reported so far in only 26 cases&#46; We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne&#46; The patient developed acute proximal weakness&#44; shoulder pain and elevated creatine-kinase &#40;CK&#41;&#46; He also developed generalized facial erythema and Gottron&#39;s papules&#46; Laboratory tests showed positive antinuclear and anti-Mi2 antibodies&#46; Further analysis confirmed CK levels above 1000<span class="elsevierStyleHsp" style=""></span>U&#47;l&#46; The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discussed&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">La asociaci&#243;n de dermatomiositis y miastenia gravis &#40;MG&#41; es infrecuente&#44; habi&#233;ndose comunicado hasta la actualidad &#250;nicamente 26 casos&#46; Se presenta el caso de un var&#243;n de 69 a&#241;os diagnosticado de MG 2 a&#241;os atr&#225;s&#44; en tratamiento con piridostigmina&#44; que inicia cuadro agudo de debilidad muscular proximal&#44; artralgias en hombros y elevaci&#243;n de creatincinasa &#40;CK&#41;&#59; as&#237; como aparici&#243;n de eritema facial generalizado y p&#225;pulas de Gottron&#46; En el estudio de laboratorio se evidenci&#243; positividad de anticuerpos antinucleares y anti-Mi2&#46; Ulteriores determinaciones de CK mostraron niveles por encima de 1&#46;000<span class="elsevierStyleHsp" style=""></span>U&#47;l&#46; Se discute el manejo cl&#237;nico de este paciente y las implicaciones terap&#233;uticas que plantea la coexistencia de ambas entidades&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Sang&#252;esa G&#243;mez C&#44; Flores Robles BJ&#44; M&#233;ndez Perles C&#44; Barbadillo C&#44; Godoy H&#44; Andr&#233;u JL&#46; Dermatomiositis y miastenia gravis&#58; una asociaci&#243;n infrecuente con implicaciones terap&#233;uticas&#46; Reumatol Clin&#46; 2015&#59;11&#58;244&#8211;246&#46;</p>"
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Article information
ISSN: 21735743
Original language: English
DOI:10.1016/j.reumae.2014.12.008
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