{"id":9320,"date":"2021-12-19T00:23:58","date_gmt":"2021-12-18T22:23:58","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/immunology\/systemic-sclerosis-symptoms-diagnosis-treatment-classification\/"},"modified":"2021-12-23T16:15:31","modified_gmt":"2021-12-23T14:15:31","slug":"systemic-sclerosis-symptoms-diagnosis-treatment-classification","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/immunology\/systemic-sclerosis-symptoms-diagnosis-treatment-classification\/","title":{"rendered":"Systemic sclerosis (symptoms, diagnosis, treatment, classification)"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">Systemic sclerosis (often used interchangeably with <strong>scleroderma<\/strong>) is a rare chronic disease of connective tissue, characterized by progressive sclerosis (hardening) of tissues including the skin, vessels, internal organs, and joints.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>The disease varies in its progression and involvement, ranging from localized cutanous lesions with slow progression, to generalized sclerosis of the skin and visceral organs, progressing rapidly.<\/li><li>The etiology is unknown, but it is thought that a dysregulation of the immune system activates fibroblasts which then deposit large amounts of collagen, leading to hardening (sclerosis).<\/li><li>More common in women.<\/li><\/ul>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Classification<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">Systemic sclerosis can be classified into 4 types:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li><strong>Morphea (localized scleroderma)<\/strong>. Isolated patches of hardened skin typically affecting the limbs and face.<\/li><li><strong>Limited cutaneous systemic sclerosis<\/strong>. A slow-progressing form known as CREST syndrome: <ul><li><strong>C<\/strong>alcinosis (formation of calcium deposits on the skin)<\/li><li><strong>R<\/strong>eynaud phenomenon<\/li><li><strong>E<\/strong>sophageal dysmobility (loss of regular esophageal activity; lower sphincter, swallowing, regurgitation, spasm)<\/li><li><strong>S<\/strong>clerodactylyl (localised thickening zand tightening of skin of fingers or toes)<\/li><li><strong>T<\/strong>elangiectasia (spider veins; dilated blood vessels appearing close to skin or mucous membrane)<\/li><\/ul><\/li><li><strong>Diffuse cutaneous systemic sclerosis<\/strong>. Generalized form with rapid progression, with both cutanous and extracutanous manifestations. <\/li><li><strong>Systemic sclerosis sine scleroderma<\/strong>. Only extracutanous manifestations (visceral involvement).<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Clinical presentation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Depending on the form, the patient may present with:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Raynaud phenomenon (secondary form) with tissue ulceration<\/li><li>Sclerosis of the skin; claw-like appearance of the hand (sclerodactylyl)<\/li><li>Arthritis<\/li><li><strong>Pulmonary involvement<\/strong>. Pulmonary fibrosis; most common cause of death<\/li><li><strong>Renal involvement<\/strong>. Renal ischemia and hypertension.<\/li><li><strong>GI involvement<\/strong>. Dysphagia and gastric reflux (leading to secondary contractures), constipation\/diarrhea<\/li><li><strong>Cardiac involvement<\/strong>. Diffuse fibrosis and conduction abnormalities.<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<p class=\"wp-block-paragraph\">EULAR\/ACR criteria is a scoring system based on the <strong>symptoms <\/strong>and<strong> serologic findings<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Serology<ul><li>Most patients appear with ANAs<\/li><\/ul><ul><li><strong>Anticentromere antibodies<\/strong> (in localized form)<\/li><li><strong>Anti-topoisomerase I<\/strong> aka anti-Scl70, and anti-RNA polymerase III (generalized form)<\/li><\/ul><\/li><li>Barium swallow test (for esophageal dysmobility)<\/li><li>Chest CT (evaluation of the lungs)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<p class=\"wp-block-paragraph\">There&#8217;s no treatment for the disease, only the symptoms.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>DMARDs (methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil)<\/li><li>Colchicine<\/li><li>Rituximab<\/li><li>PPIs (reflux disease)<\/li><li>ACEi\/ARBs (renal HTN)<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Classification<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Clinical presentation<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"<p>Systemic sclerosis (often used interchangeably with scleroderma) is a rare chronic disease of connective tissue, characterized by progressive sclerosis (hardening) of tissues including the skin, vessels, internal organs, and joints. The disease varies in its progression and involvement, ranging from localized cutanous lesions with slow progression, to generalized sclerosis of the skin and visceral organs, [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":9306,"menu_order":6,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-9320","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.9 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Systemic sclerosis (symptoms, diagnosis, treatment, classification) &#8211; Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/immunology\/systemic-sclerosis-symptoms-diagnosis-treatment-classification\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/immunology\\\/systemic-sclerosis-symptoms-diagnosis-treatment-classification\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/immunology\\\/systemic-sclerosis-symptoms-diagnosis-treatment-classification\\\/\",\"name\":\"Systemic sclerosis (symptoms, diagnosis, treatment, classification) &#8211; 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