{"id":2513,"date":"2009-03-08T11:55:50","date_gmt":"2009-03-08T11:55:50","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/reuss\/"},"modified":"2025-05-14T19:10:27","modified_gmt":"2025-05-14T19:10:27","slug":"reuss","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/reuss\/","title":{"rendered":"Criteria for the Classification of Systemic Sclerosis (Scleroderma)"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p><\/p>\n<p class=\"MsoNormal\"><strong> 1980 Criteria for the Classification of Systemic Sclerosis<\/strong><\/p>\n<p class=\"MsoNormal\">The American College of Rheumatology (former American Rheumatism Association &#8211; ARA) has defined criteria, that are 97 % sensitive and 98 % specific for systemic sclerosis (SSc) as follows:<\/p>\n<p class=\"MsoNormal\"><strong>Major criterion: <\/strong><\/p>\n<ul>\n<li class=\"MsoNormal\">Proximal diffuse (truncal) sclerosis (skin tightness, thickening, non-pitting induration)<\/li>\n<\/ul>\n<p><!--more--><br \/>\n<strong>Minor criteria:<\/strong><\/p>\n<ul>\n<li>\n<p class=\"MsoNormal\" style=\"margin-top: 0pt; margin-bottom: 0pt;\">Sclerodactyly (only fingers and\/or toes)<\/p>\n<\/li>\n<li>\n<p class=\"MsoNormal\" style=\"margin-top: 0pt; margin-bottom: 0pt;\">Digital pitting scars or loss of substance of the digital finger pads (pulp loss)<\/p>\n<\/li>\n<li>\n<p class=\"MsoNormal\" style=\"margin-top: 0pt; margin-bottom: 0pt;\">Bilateral basilar pulmonary fibrosis<\/p>\n<\/li>\n<\/ul>\n<p class=\"MsoNormal\">The patient should fulfill the major criterion or two of the three minor criteria. Raynaud&#8217;s phenomenon is observed in 90-98 % of SSc patients.<\/p>\n<p class=\"MsoNormal\"><strong>Subsets of Systemic Sclerosis<\/strong><\/p>\n<table style=\"border-collapse: collapse; width: 100%;\" border=\"1\">\n<tbody>\n<tr>\n<td style=\"border: 1pt solid windowtext; padding: 0cm 5.4pt; width: 125px;\" valign=\"top\">\n<p class=\"MsoNormal\">\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 178px; border: 1pt 1pt 1pt medium solid solid solid none windowtext windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\"><strong> Diffuse<\/strong><\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 176px; border: 1pt 1pt 1pt medium solid solid solid none windowtext windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\"><strong> Limited*<\/strong><\/p>\n<\/td>\n<\/tr>\n<tr>\n<td style=\"padding: 0cm 5.4pt; width: 125px; border: medium 1pt 1pt none solid solid -moz-use-text-color windowtext windowtext;\" valign=\"top\">\n<p class=\"MsoNormal\">Skin involvement<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 178px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Distal and proximal<\/p>\n<p class=\"MsoNormal\">extremities, face, trunk<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 176px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Distal to elbows, face<\/p>\n<\/td>\n<\/tr>\n<tr>\n<td style=\"padding: 0cm 5.4pt; width: 125px; border: medium 1pt 1pt none solid solid -moz-use-text-color windowtext windowtext;\" valign=\"top\">\n<p class=\"MsoNormal\">Raynaud\u2019s phenomenon<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 178px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Onset within 1 year or at time of skin changes<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 176px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">May precede skin disease by years<\/p>\n<\/td>\n<\/tr>\n<tr>\n<td style=\"padding: 0cm 5.4pt; width: 125px; border: medium 1pt 1pt none solid solid -moz-use-text-color windowtext windowtext;\" valign=\"top\">\n<p class=\"MsoNormal\">Organ involvement<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 178px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Pulmonary (interstitial fibrosis); renal (renovascular hypertensive crisis); gastrointestinal; cardiac<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 176px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Gastrointestinal; pulmonary arterial hypertension after 10-15 years of disease in &lt;10% of patients; biliary cirrhosis<\/p>\n<\/td>\n<\/tr>\n<tr>\n<td style=\"padding: 0cm 5.4pt; width: 125px; border: medium 1pt 1pt none solid solid -moz-use-text-color windowtext windowtext;\" valign=\"top\">\n<p class=\"MsoNormal\">Nail fold capillaries<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 178px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Dilatation and dropout<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 176px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Dilatation without significant dropout<\/p>\n<\/td>\n<\/tr>\n<tr>\n<td style=\"padding: 0cm 5.4pt; width: 125px; border: medium 1pt 1pt none solid solid -moz-use-text-color windowtext windowtext;\" valign=\"top\">\n<p class=\"MsoNormal\">Antinuclear antibodies<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 178px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Anti-topoisomerase 1<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 176px; border: medium 1pt 1pt medium none solid solid none -moz-use-text-color windowtext windowtext -moz-use-text-color;\" valign=\"top\">\n<p class=\"MsoNormal\">Anticentromere<\/p>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p class=\"MsoNormal\">* Also referred to as CREST( calcinosis, Raynaud\u2019s, esophageal dysmotility, sclerodactyly, telangiectasia).<\/p>\n<p class=\"MsoNormal\"><strong> ABCDCREST Criteria for the Classification of Systemic Sclerosis<\/strong><\/p>\n<ol>\n<li class=\"MsoNormal\">Autoantibodies: autoantibodies to centromere proteins (CENPs) detected by indirect immunofluorescence; anti-Scl-70 (topoisomerase I) detected by double immunodiffusion; anti-fibrillarin (U3-RNP) detected by immunoprecipitation<\/li>\n<li class=\"MsoNormal\">Bibasilar pulmonary fibrosis detected by chest radiograph: linear shadows or \u201choney-comb\u201d reticular appearance most expressed at the periphery of the lungs and at the bases<\/li>\n<li class=\"MsoNormal\">Contracture of the joints defined as permanent limitation of joint motion. The prayer sign is detected when a patient opposed the palmar surfaces of both hands with extended wrists. The sign is positive when the patient is unable to oppose the palms. This suggests joint or skin pathology, or shortening of the forearm flexors<\/li>\n<li class=\"MsoNormal\">Dermal thickening can be defined by the modified Rodnan skin score, which employs clinical palpation of the skin as described<\/li>\n<li class=\"MsoNormal\">Calcinosis cutis, most often located on the fingers, is intra and\/or subcutaneous deposits of hydroxyapatite that can ulcerate the skin; it can be detected by radiography, crystallographic or chemical analysis<\/li>\n<li class=\"MsoNormal\">Raynaud\u2019s phenomenon is a sudden pallor of an acral structure (e.g., fingers, whole hand, toes, tip of nose, earlobe, or tongue). The involved area may subsequently develop cyanosis and, with re-warming, become erythematous. Determination is by patient\u2019s history or physician\u2019s observation<\/li>\n<li class=\"MsoNormal\">Esophageal distal hypomotility can be detected by cine\/video barium esophagram, performed in the upright and supine position. Reflux-esophagitis can be detected by esophagogastroduodenoscopy in the forms of erosive esophagitis or Barret\u2019s esophagus<\/li>\n<li class=\"MsoNormal\">Sclerodactyly is symmetric thickening and tightening of the skin on the digits. Before sclerodactyly develops, there could be a phase of non-pitting digital edema of varying duration. It is defined as non-pitting increase in soft tissue mass of the digits that extends beyond the normal confines of the joint capsules<\/li>\n<li class=\"MsoNormal\">Teleangiectasias are visible macular dilatations of superficial cutaneous blood vessels that collapse upon pressure and fill slowly when pressure is released. Common locations are the digits, face, lips, tongue<\/li>\n<\/ol>\n<p class=\"MsoNormal\">A classification of definite SSc requires three or more criteria.<\/p>\n<p>&nbsp;<\/p>\n<p class=\"MsoNormal\"><strong>References:<\/strong><\/p>\n<ol>\n<li class=\"MsoNormal\">Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980;23:581-90. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/7378088\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<li class=\"MsoNormal\">Nadashkevich O, Davis P, Fritzler MJ. A proposal of criteria for the classification of systemic sclerosis. Med. Sci. Monit. 2004 Nov;10(11):CR615-21. Epub 2004 Oct 26. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/15507853\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created: Jul 20, 2007<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[45],"tags":[166,167,16,15,1573,94,1070,1572,93,1571,1088],"class_list":["post-2513","post","type-post","status-publish","format-standard","hentry","category-rheumatology","tag-clasificacion","tag-classification","tag-criteria","tag-criterios","tag-escleroderma","tag-esclerosis","tag-rheumatology","tag-scleroderma","tag-sclerosis","tag-sistemica","tag-systemic"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2513","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=2513"}],"version-history":[{"count":3,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2513\/revisions"}],"predecessor-version":[{"id":9149,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2513\/revisions\/9149"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=2513"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=2513"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=2513"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}