{"id":8381,"date":"2021-01-29T21:07:24","date_gmt":"2021-01-29T21:07:24","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=8381"},"modified":"2026-01-06T20:45:38","modified_gmt":"2026-01-06T20:45:38","slug":"igg4-sc","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/igg4-sc\/","title":{"rendered":"Clinical Diagnostic Criteria of IgG4-Related Sclerosing Cholangitis (IgG4-SC)"},"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>Sclerosing cholangitis (SC) is defined as a condition with progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis and currently includes three categories: primary sclerosing cholangitis (PSC), secondary cholangitis, and IgG4-related sclerosing cholangitis (IgG4-SC). SC categories share similar clinical features, such as cholestasis. Patients with SC present with cholestatic symptoms, including jaundice and pruritus, and blood tests reveal elevation of cholestatic enzymes. <!--more--><\/p>\n<p><strong>Clinical Diagnostic Criteria of IgG4-Related Sclerosing Cholangitis<\/strong><\/p>\n<p><strong>Diagnostic items<\/strong><\/p>\n<ol>\n<li>Biliary tract imaging reveals diffuse or segmental narrowing of the intrahepatic and\/or extrahepatic bile duct, associated with thickening of the bile duct wall<\/li>\n<li>Hematological examination shows elevated serum IgG4 concentration (\u2265135 mg\/dL)<\/li>\n<li>Coexistence of <a href=\"https:\/\/medicalcriteria.com\/web\/autoimmune-pancreatitis\/\" target=\"_blank\" rel=\"noopener noreferrer\">autoimmune pancreatitis<\/a>, IgG4-related dacryoadenitis\/sialadenitis, or IgG4-related retroperitoneal fibrosis<\/li>\n<li>Histopathological examination shows:<\/li>\n<\/ol>\n<ol>\n<li style=\"list-style-type: none;\">\n<ol style=\"list-style-type: lower-alpha;\">\n<li>Marked lymphocytic and plasmacytic infiltration and fibrosis<\/li>\n<li>Infiltration of IgG4-positive plasma cells (&gt;10 cells per high power field)<\/li>\n<li>Storiform fibrosis<\/li>\n<li>Obliterative phlebitis<\/li>\n<\/ol>\n<\/li>\n<\/ol>\n<p><strong>Optional:<\/strong> effectiveness of steroid therapy<\/p>\n<p><strong>Diagnosis<\/strong><\/p>\n<ul>\n<li>Definite diagnosis: (1)+(3), or (1)+(2)+(4) (a+b, or a+b+c, or a+b+d)<\/li>\n<li>Probable diagnosis: (1)+(2)+optional item<\/li>\n<li>Possible diagnosis: (1)+(2)<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Tanaka A. IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis. Gut Liver. 2019 May 15;13(3):300-307. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/30205418\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Tanaka A. Immunoglobulin G4-related sclerosing cholangitis. J Dig Dis. 2019 Jul;20(7):357-362. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/31112324\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Naitoh I, Yoshida M, Nakazawa T. Endoscopic diagnosis of immunoglobulin G4-related sclerosing cholangitis. Dig Endosc. 2025 Aug;37(8):824-833. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/40256978\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created: Jan 27, 2021.<br \/>\nUp-date: Jan 06, 2026.<\/p>\n<p>&nbsp;<\/p>\n<p>&nbsp;<\/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":[53],"tags":[376,490,489,375,16,15,14,13,660,2308,2309,1563,662],"class_list":["post-8381","post","type-post","status-publish","format-standard","hentry","category-gastroenterology","tag-cholangitis","tag-clinical","tag-clinico","tag-colangitis","tag-criteria","tag-criterios","tag-diagnostic","tag-diagnostico","tag-esclerosante","tag-igg4","tag-relacionada","tag-related","tag-sclerosing"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/8381","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=8381"}],"version-history":[{"count":8,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/8381\/revisions"}],"predecessor-version":[{"id":10566,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/8381\/revisions\/10566"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=8381"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=8381"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=8381"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}