{"id":7009,"date":"2020-04-24T03:02:20","date_gmt":"2020-04-24T06:02:20","guid":{"rendered":"https:\/\/doctorhoogstra.com\/es\/sindrome-antisintetasa-2\/"},"modified":"2020-04-27T16:33:32","modified_gmt":"2020-04-27T19:33:32","slug":"syndrome-antisynthetase-2","status":"publish","type":"wiki","link":"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/","title":{"rendered":"Anti-synthase syndrome"},"content":{"rendered":"<p><\/p><div id=\"ez-toc-container\" class=\"ez-toc-v2_0_88 counter-hierarchy ez-toc-counter ez-toc-grey ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Contents<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 eztoc-toggle-hide-by-default' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#%C2%BFQue-es-la-antisintetasa-sindrome\" >What is antisynthetase? syndrome?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#%C2%BFQue-causa-el-sindrome-antisintetasa\" >What causes antisynthetase syndrome?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#%C2%BFComo-se-presenta-el-sindrome-antisintetasa\" >How does antisynthetase syndrome present?<\/a><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><ul class='ez-toc-list-level-5' ><li class='ez-toc-heading-level-5'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#Sindrome-antisintetasa\" >Anti-synthase syndrome<\/a><\/li><\/ul><\/li><\/ul><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#Asociacion-con-malignidad\" >Association with malignancy<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#%C2%BFComo-se-hace-el-diagnostico-del-sindrome-antisintetasa\" >How is the diagnosis of antisynthetase syndrome made?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#%C2%BFComo-se-trata-el-sindrome-antisintetasa\" >How is antisynthetase syndrome treated?<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/doctorhoogstra.com\/en\/wiki\/sindrome-antisintetasa-2\/#Pronostico-del-sindrome-antisintetasa\" >Antisynthetase Syndrome Prognosis<\/a><\/li><\/ul><\/nav><\/div>\n\n<section class=\"textBlock\">\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-es-la-antisintetasa-sindrome\"><\/span>What is antisynthetase? <span class=\"term\" data-term-id=\"350\">syndrome<\/span>?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Antisynthetase syndrome is a rare <span class=\"term\" data-term-id=\"490\">inflammatory<\/span> muscle disease related to dermatomyositis and polymyositis.<\/p>\n<p>The hallmark of antisynthetase syndrome is the presence of <span class=\"term\" data-term-id=\"1430\">serum<\/span> <span class=\"term\" data-term-id=\"352\">autoantibodies<\/span> directed against aminoacyl-tRNA synthetases. These are <span class=\"term\" data-term-id=\"956\">cellular<\/span> <span class=\"term\" data-term-id=\"1526\">enzymes<\/span> involved in protein synthesis. antisynthetase <span class=\"term\" data-term-id=\"426\">antibodies<\/span> they include Jo-1, PL-7, PL-12, OJ, EJ, KS, Wa, YRS, and Zo. Anti-Jo-1 antibodies are the most commonly detected in antisynthetase syndrome.<\/p>\n<p>Antisynthetase syndrome is 2\u20133 times more common in women than in men.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFQue-causa-el-sindrome-antisintetasa\"><\/span>What causes antisynthetase syndrome?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The cause of antisynthetase syndrome is unknown. It is classified as a <span class=\"term\" data-term-id=\"319\">chronic<\/span> <span class=\"term\" data-term-id=\"691\">autoimmune<\/span> disease.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-presenta-el-sindrome-antisintetasa\"><\/span>How does antisynthetase syndrome present?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The main clinical features of antisynthetase syndrome are:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"1176\">Fever<\/span><\/li>\n<li>\n<span class=\"term\" data-term-id=\"1802\">myositis<\/span> (muscle <span class=\"term\" data-term-id=\"148\">inflammation<\/span>)<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1548\">Polyarthritis<\/span> (inflammation in various joints)<\/li>\n<li>\n<span class=\"term\" data-term-id=\"750\">Interstitial<\/span> lung disease (non-specific inflammation of the lungs)<\/li>\n<li>mechanic hands<\/li>\n<li><span class=\"term\" data-term-id=\"256\">Raynaud&#039;s phenomenon<\/span><\/li>\n<\/ul>\n<p>Antisynthetase syndrome can present with a variety of clinical features and these can vary over time.<\/p>\n<p>Fever<\/p>\n<ul>\n<li>Present in approximately 20% of patients<\/li>\n<li>It can occur early in the disease.<\/li>\n<li>May persist or recur <span class=\"term\" data-term-id=\"1501\">relapses<\/span>\n<\/li>\n<\/ul>\n<p>myositis<\/p>\n<ul>\n<li>Present in &gt;90% of patients<\/li>\n<li>Associated with anti Jo-1 antibodies<\/li>\n<li>\n<span class=\"term\" data-term-id=\"242\">Proximal<\/span> muscle weakness causes difficulty getting up from a chair or climbing stairs<\/li>\n<li>The muscles can be sore.<\/li>\n<li>Weakness of the muscles involved in swallowing can cause <span class=\"term\" data-term-id=\"1867\">aspiration<\/span> pneumonia.<\/li>\n<li>Weakness of the respiratory muscles can cause shortness of breath.<\/li>\n<\/ul>\n<p>Inflammatory <span class=\"term\" data-term-id=\"1578\">arthritis<\/span><\/p>\n<ul>\n<li>50% of patients experience joint pain or arthritis<\/li>\n<li>More often <span class=\"term\" data-term-id=\"279\">symmetrical<\/span> arthritis of small joints of the hands and feet<\/li>\n<li>Usually does not result in bone <span class=\"term\" data-term-id=\"536\">erosions<\/span>\n<\/li>\n<\/ul>\n<p>Lung disease<\/p>\n<ul>\n<li>Interstitial lung disease develops in most patients with anti-Jo-1 antisynthetase syndrome.<\/li>\n<li>Often presents with sudden or gradual onset of shortness of breath with exercise<\/li>\n<li>Sometimes causes intractable dry cough<\/li>\n<li>Can lead to <span class=\"term\" data-term-id=\"1009\">pulmonary<\/span> <span class=\"term\" data-term-id=\"561\">hypertension<\/span> (increased pressure in the pulmonary arteries) in patients with or without <span class=\"term\" data-term-id=\"912\">concomitant<\/span> interstitial lung disease.<\/li>\n<\/ul>\n<p>mechanic hands<\/p>\n<ul>\n<li>It affects approximately 30% of patients.<\/li>\n<li>Thickened skin of the tips and margins of the fingers.<\/li>\n<li>It resembles the hands of a mechanic.<\/li>\n<\/ul>\n<p>Raynaud&#039;s phenomenon<\/p>\n<ul>\n<li>It occurs in approximately 40% of patients.<\/li>\n<li>An episodic reduction in the blood supply of the fingers and toes that turn white, then blue, and finally red<\/li>\n<li>A response to cold or emotional stress.<\/li>\n<li>Some patients have associated nail fold <span class=\"term\" data-term-id=\"756\">capillary<\/span> abnormalities<\/li>\n<\/ul>\n<p>Symptoms of antisynthetase syndrome<\/p>\n<section class=\"textBlock imageLinkBlockTitle\">\n<h5 class=\"colour--primary\"><span class=\"ez-toc-section\" id=\"Sindrome-antisintetasa\"><\/span>Anti-synthase syndrome<span class=\"ez-toc-section-end\"><\/span><\/h5>\n<\/section>\n<section class=\"imageLinkBlock\">\n<div class=\"flex\">\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='antisynthetase-1__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-8631452-5140256' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/antisynthetase-1__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-8631452.jpg'>\n<\/div>\n<p class=\"p-small\">Capillary damage of the nail fold in antisynthetase syndrome<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='antisynthetase-5__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-2629966-4014705' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/antisynthetase-5__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-2629966.jpg'>\n<\/div>\n<p class=\"p-small\">Anti-synthase syndrome<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='antisynthetase-4__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-5195457-7183806' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/antisynthetase-4__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-5195457.jpg'>\n<\/div>\n<p class=\"p-small\">Anti-synthase syndrome<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='antisynthetase-capillaries-01__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-7255135-6397743' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/antisynthetase-capillaries-01__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-7255135.jpg'>\n<\/div>\n<p class=\"p-small\">Abnormal capillaries of the nail fold<\/p>\n<div class=\"imageLinkBlock__item__image\">\n<p>                        <img alt='antisynthetase-capillaries-02__protectwyjqcm90zwn0il0_focusfillwzi5ncwymjisingildfd-7570912-8388573' src='https:\/\/doctorhoogstra.com\/wp-content\/uploads\/2020\/04\/antisynthetase-capillaries-02__ProtectWyJQcm90ZWN0Il0_FocusFillWzI5NCwyMjIsIngiLDFd-7570912.jpg'>\n<\/div>\n<p class=\"p-small\">Note petechiae on cuticle<\/p>\n<\/p><\/div>\n<\/section>\n<div class=\"clearfix imageLinkBlock\"><\/div>\n<h2><span class=\"ez-toc-section\" id=\"Asociacion-con-malignidad\"><\/span>Association with <span class=\"term\" data-term-id=\"1742\">malignancy<\/span><br \/>\n<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Some case studies have reported various malignancies occurring within 6 to 12 months of diagnosis of antisynthetase syndrome. Therefore, age-appropriate screening is recommended, as for dermatomyositis.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-hace-el-diagnostico-del-sindrome-antisintetasa\"><\/span>How is the diagnosis of antisynthetase syndrome made?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The clinical presentation is a clue to the diagnosis of antisynthetase syndrome. Special investigations help support the diagnosis. These may include the following depending on the clinical context:<\/p>\n<ul>\n<li>Muscle enzymes, for example, creatinine kinase (CK) and aldolase: these are often elevated<\/li>\n<li>muscle antibodies<\/li>\n<li>Electromyography (EMG)<\/li>\n<li>\n<span class=\"term\" data-term-id=\"1410\">Magnetic resonance imaging<\/span> (<span class=\"term\" data-term-id=\"1116\">Magnetic resonance<\/span>) of the affected muscles<\/li>\n<li>Muscle <span class=\"term\" data-term-id=\"24\">biopsy<\/span>\n<\/li>\n<li>Lung function tests<\/li>\n<li>High resolution <span class=\"term\" data-term-id=\"797\">computed tomography<\/span> scan (<span class=\"term\" data-term-id=\"799\">Connecticut<\/span>) of the chest<\/li>\n<li>Assessment of swallowing difficulties and risk of aspiration.<\/li>\n<li>lung biopsy<\/li>\n<\/ul>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n<div id=\"dermnet-dermnet-mobbox\">\n<\/div><\/div><\/div><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%C2%BFComo-se-trata-el-sindrome-antisintetasa\"><\/span>How is antisynthetase syndrome treated?<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><span class=\"term\" data-term-id=\"1429\">Glucocorticosteroids<\/span> they are the mainstay of treatment for antisynthetase syndrome and are often required for several months or years. Prednisone is initially given in high doses (1 mg\/kg\/day) for 4 to 6 weeks to achieve disease <span class=\"term\" data-term-id=\"1854\">control<\/span>, then tapered slowly over 9-12 months to the lowest effective dose to maintain <span class=\"term\" data-term-id=\"1260\">remission<\/span>. In more severe cases, pulsed intravenous (IV) methylprednisolone for 3-5 days may be necessary.<\/p>\n<p>Improvement in muscle strength can take several weeks or months. Symptomatic improvement is a more reliable indicator of response to treatment than serum CK levels.<\/p>\n<p><span class=\"term\" data-term-id=\"1738\">Prophylactic<\/span> Treatment for steroid-induced osteoporosis and certain fungal infections such as Pneumocystis jirovecii is recommended. The need for vaccinations should be evaluated before starting therapy.<\/p>\n<p>Other immunosuppressive drugs may be used, such as:<\/p>\n<ul>\n<li>Azathioprine<\/li>\n<li>Methotrexate<\/li>\n<li>Cyclophosphamide<\/li>\n<li>Tacrolimus<\/li>\n<li>Cyclosporine<\/li>\n<li>Mycophenolate<\/li>\n<li>Rituximab<\/li>\n<\/ul>\n<p>Physical therapy and rehabilitation should begin early to prevent muscle deconditioning and improve weakness.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"Pronostico-del-sindrome-antisintetasa\"><\/span>\n<span class=\"term\" data-term-id=\"241\">Forecast<\/span> of antisynthetase syndrome<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Patients with antisynthetase syndrome respond well to <span class=\"term\" data-term-id=\"380\">systemic<\/span> steroids and\/or immunosuppressive agents if they only have muscle involvement.<\/p>\n<p>If the lungs are involved, the severity and type of lung injury determine the prognosis. Advanced age at onset (&gt;60 years), presence of malignancy, and negative ANA <span class=\"term\" data-term-id=\"425\">antibody<\/span> the test confers a worse prognosis.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es la antisintetasa? s\u00edndrome? El s\u00edndrome de antisintetasa es un raro inflamatorio enfermedad muscular relacionada con dermatomiositis y polimiositis. El sello distintivo del s\u00edndrome antisintetasa es la presencia de&#8230;<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-7009","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7009","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/comments?post=7009"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7009\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=7009"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=7009"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=7009"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}