{"id":8293,"date":"2021-01-14T21:02:01","date_gmt":"2021-01-14T21:02:01","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=8293"},"modified":"2026-01-06T20:47:27","modified_gmt":"2026-01-06T20:47:27","slug":"igg4-rd","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/es\/igg4-rd\/","title":{"rendered":"Criterios de Clasificaci\u00f3n para la Enfermedad Relacionada con IgG4 (ER-IgG4)"},"content":{"rendered":"<div class=\"7ca9444b8380138fe077da07353677a4\" 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>La enfermedad relacionada con la inmunoglobulina G4 (ER-IgG4) es una afecci\u00f3n inmunomediada que produce enfermedades en varios \u00f3rganos del cuerpo como el p\u00e1ncreas, los ri\u00f1ones, las gl\u00e1ndulas salivales, los pulmones, el h\u00edgado, los ganglios linf\u00e1ticos, el tracto biliar y las \u00f3rbitas de los ojos. La enfermedad se reconoce por un patr\u00f3n caracter\u00edstico de caracter\u00edsticas patol\u00f3gicas, serol\u00f3gicas o cl\u00ednicas compartidas entre los sistemas de \u00f3rganos involucrados. En algunos casos, ER-IgG4 puede simular trastornos malignos, infecciosos o inflamatorios y, por lo tanto, distinguir la enfermedad es crucial.<!--more--><br \/>\nEl consenso internacional sobre ER-IgG4 estableci\u00f3 las bases para el diagn\u00f3stico de ER-IgG4 a partir de hallazgos en m\u00faltiples \u00f3rganos. El algoritmo definitivo para el diagn\u00f3stico de ER-IgG4 incluye: inflamaci\u00f3n de \u00f3rganos (agrandamiento, masa o lesiones nodulares) o disfunci\u00f3n org\u00e1nica en uno o m\u00e1s \u00f3rganos en el examen f\u00edsico; estudios hematol\u00f3gicos que muestren una concentraci\u00f3n s\u00e9rica de IgG4+ \u2265 135 mg\/dL; histopatolog\u00eda que indica infiltraci\u00f3n marcada de linfocitos y plasmocitos con fibrosis e infiltraci\u00f3n de c\u00e9lulas plasm\u00e1ticas IgG4+ (IgG4+\/c\u00e9lulas IgG + totales &gt; 40% Y &gt; 10 c\u00e9lulas IgG4+ en campo de alta potencia).<\/p>\n<p><strong>Criterios de Clasificaci\u00f3n para la Enfermedad Relacionada con IgG4<\/strong><\/p>\n<p><strong>1) Caracter\u00edsticas cl\u00ednicas y radiol\u00f3gicas<\/strong><br \/>\nUno o m\u00e1s \u00f3rganos muestran hinchaz\u00f3n difusa o localizada o una masa o n\u00f3dulo caracter\u00edstico de IgG4-RD. En afectaci\u00f3n de un simple \u00f3rgano, se omite la inflamaci\u00f3n de los ganglios linf\u00e1ticos.<\/p>\n<p><strong>2) Diagn\u00f3stico serol\u00f3gico<\/strong><br \/>\nNiveles s\u00e9ricos de IgG4 superiores a 135 mg\/dl.<\/p>\n<p><strong>3) Diagn\u00f3stico patol\u00f3gico<\/strong><br \/>\nPositividad para dos de los siguientes tres criterios:<\/p>\n<ol>\n<li>Infiltraci\u00f3n de linfocitos densos y c\u00e9lulas plasm\u00e1ticas con fibrosis.<\/li>\n<li>Relaci\u00f3n de c\u00e9lulas plasm\u00e1ticas positivas para IgG4\/c\u00e9lulas positivas para IgG superior al 40% y el n\u00famero de IgG4-c\u00e9lulas plasm\u00e1ticas positivas superiores a 10 por campo de alta potencia<\/li>\n<li>Fibrosis tisular t\u00edpica, en particular fibrosis estoriforme o flebitis obliterante<\/li>\n<\/ol>\n<p><strong>Diagn\u00f3stico:<\/strong><\/p>\n<ul>\n<li>Definido: 1) +2) +3)<\/li>\n<li>Probable: 1) +3)<\/li>\n<li>Posible: 1) +2)<\/li>\n<\/ul>\n<p><strong>Nota explicativa 1:<\/strong> Combinaci\u00f3n de criterios de diagn\u00f3stico espec\u00edficos de \u00f3rganos *.<br \/>\nPacientes con un diagn\u00f3stico posible o probable mediante criterios diagn\u00f3sticos integrales que cumplan los requisitos espec\u00edficos del \u00f3rgano.<br \/>\nLos criterios para IgG4-RD se consideran definitivos para IgG4-RD.<\/p>\n<p>* Criterios diagn\u00f3sticos seg\u00fan el \u00f3rgano relacionado con IgG4:<\/p>\n<ol>\n<li><a href=\"https:\/\/medicalcriteria.com\/web\/es\/autoimmune-pancreatitis\/\" target=\"_blank\" rel=\"noopener noreferrer\">Criterios diagn\u00f3sticos de consenso internacional para pancreatitis autoinmune<\/a><\/li>\n<li>Gl\u00e1ndula lagrimal relacionada con IgG4, criterios diagn\u00f3sticos de adenitis salival<\/li>\n<li><a href=\"https:\/\/medicalcriteria.com\/web\/es\/igg4-rkd\/\" target=\"_blank\" rel=\"noopener noreferrer\">Criterios diagn\u00f3stico para la enfermedad renal relacionada con IgG4<\/a><\/li>\n<li><a href=\"https:\/\/medicalcriteria.com\/web\/es\/igg4-sc\/\" target=\"_blank\" rel=\"noopener noreferrer\">Criterios diagn\u00f3stico cl\u00ednico de la colangitis esclerosante relacionada con IgG4<\/a><\/li>\n<li>Criterios diagn\u00f3stico para la enfermedad oft\u00e1lmica relacionada con IgG4<\/li>\n<li>Criterios diagn\u00f3stico de enfermedad respiratoria relacionada con IgG4<\/li>\n<li>Criterios diagn\u00f3stico para periarteritis grande\/periarteritis y fibrosis retroperitoneal relacionada con IgG4<\/li>\n<\/ol>\n<p><strong>Nota explicativa 2: diagn\u00f3stico de exclusi\u00f3n<\/strong><br \/>\n1) Es importante adquirir muestras de tejido de cada \u00f3rgano involucrado para distinguir los tumores malignos (por ejemplo, c\u00e1ncer, linfoma maligno) y afecciones benignas similares (por ejemplo, s\u00edndrome de Sj\u00f6gren, colangitis esclerosante primaria, enfermedad de Castleman multic\u00e9ntrica, fibrosis retroperitoneal secundaria, granulomatosis con poliangitis, sarcoidosis, granulomatosis eosinof\u00edlica con poliange\u00edtis).<br \/>\n2) Es importante excluir una enfermedad infecciosa o relacionada con la inflamaci\u00f3n en pacientes con fiebre alta, muy PCR elevada y neutrofilia.<\/p>\n<p><strong>Nota explicativa 3: diagn\u00f3stico patol\u00f3gico<\/strong><br \/>\n1) El n\u00famero de c\u00e9lulas positivas para IgG4 suele ser m\u00e1s abundante en \u00f3rganos resecados y tejido parcialmente enucleado que en muestras de tejido obtenidas mediante biopsia con aguja o biopsia endosc\u00f3pica.<br \/>\n2) La fibrosis estoriforme se define como c\u00e9lulas fusiformes, c\u00e9lulas inflamatorias y fibras de col\u00e1geno finas que confluyen. La flebitis obliterante se define como la obliteraci\u00f3n venosa fibrosa con c\u00e9lulas inflamatorias. Ambos son \u00fatil para el diagn\u00f3stico de IgG4-RD. 1 y 3 sin 2 solo se pueden aplicar en caso de tinci\u00f3n deficiente de IgG4 y\/o IgG.<\/p>\n<p><strong>Nota explicativa 4: reactividad a los esteroides<\/strong><br \/>\nNo se recomienda la prueba de esteroides. Sin embargo, si los pacientes no responden a la terapia inicial con esteroides, se debe reconsiderar el diagn\u00f3stico.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>Bibliograf\u00eda:<\/strong><\/p>\n<ol>\n<li>Lanzillotta M, Mancuso G, Della-Torre E. Advances in the diagnosis and management of IgG4 related disease. BMJ. 2020 Jun 16;369:m1067. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/32546500\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Umehara H, Okazaki K, Kawa S, Takahashi H, Goto H, Matsui S, Ishizaka N, Akamizu T, Sato Y, Kawano M; Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW) Japan. The 2020 Revised Comprehensive Diagnostic (RCD) Criteria for IgG4-RD. Mod Rheumatol. 2020 Dec 4:1-14. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/33274670\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Vasaitis L. IgG4-related disease: A relatively new concept for clinicians. Eur J Intern Med. 2016 Jan;27:1-9. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/26481243\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Creado Ene 7, 2021.<\/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>La enfermedad relacionada con la inmunoglobulina G4 (ER-IgG4) es una afecci\u00f3n inmunomediada que produce enfermedades en varios \u00f3rganos del cuerpo como el p\u00e1ncreas, los ri\u00f1ones, las gl\u00e1ndulas salivales, los pulmones, el h\u00edgado, los ganglios linf\u00e1ticos, el tracto biliar y las \u00f3rbitas de los ojos. La enfermedad se reconoce por un patr\u00f3n caracter\u00edstico de caracter\u00edsticas patol\u00f3gicas, [&hellip;]<\/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":[166,167,16,15,51,50,2308,2309,1563],"class_list":["post-8293","post","type-post","status-publish","format-standard","hentry","category-rheumatology","tag-clasificacion","tag-classification","tag-criteria","tag-criterios","tag-disease","tag-enfermedad","tag-igg4","tag-relacionada","tag-related"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.1 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Criterios de Clasificaci\u00f3n para la Enfermedad Relacionada con IgG4 (ER-IgG4) - MedicalCRITERIA.com<\/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:\/\/medicalcriteria.com\/web\/es\/igg4-rd\/\" \/>\n<meta property=\"og:locale\" content=\"es_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"[:es]Criterios de Clasificaci\u00f3n para la Enfermedad Relacionada con IgG4 (ER-IgG4)[:en]Classification Criteria for IgG4-Related Disease[:] - MedicalCRITERIA.com\" \/>\n<meta property=\"og:description\" content=\"La enfermedad relacionada con la inmunoglobulina G4 (ER-IgG4) es una afecci\u00f3n inmunomediada que produce enfermedades en varios \u00f3rganos del cuerpo como el p\u00e1ncreas, los ri\u00f1ones, las gl\u00e1ndulas salivales, los pulmones, el h\u00edgado, los ganglios linf\u00e1ticos, el tracto biliar y las \u00f3rbitas de los ojos. 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