{"id":4628,"date":"2019-05-21T09:00:19","date_gmt":"2019-05-21T09:00:19","guid":{"rendered":"https:\/\/endoscopiaterapeutica.net\/pt\/uncategorized\/pancreatite-autoimune\/"},"modified":"2023-08-07T21:28:25","modified_gmt":"2023-08-07T21:28:25","slug":"pancreatite-autoimune","status":"publish","type":"post","link":"https:\/\/endoscopiaterapeutica.net\/pt\/assuntosgerais\/pancreatite-autoimune\/","title":{"rendered":"Pancreatite Autoimune"},"content":{"rendered":"<p>\u00a0<\/p>\n<p>A Pancreatite Autoimune (PAI) \u00e9 uma das poss\u00edveis causas de pancreatite cr\u00f4nica, que cursa com infiltrado inflamat\u00f3rio na gl\u00e2ndula e fibrose progressiva, podendo levar \u00e0 insufici\u00eancia pancre\u00e1tica (1).<\/p>\n<h5>\u00a0<\/h5>\n<p><strong>A observa\u00e7\u00e3o do quadro cl\u00ednico nos permite classificar a PAI em 2 subtipos (2,3):<\/strong><\/p>\n<ul>\n<li><strong>Pancreatite Autoimune tipo 1:<\/strong> o envolvimento pancre\u00e1tico \u00e9 parte de uma condi\u00e7\u00e3o sist\u00eamica, que acomete diversos \u00f3rg\u00e3os, relacionada a infiltra\u00e7\u00e3o por c\u00e9lulas imunes ricas em IgG4 (uma sub-fra\u00e7\u00e3o da IgG). A principal caracter\u00edstica \u00e9 o inflitrado linfo-plasmocit\u00e1rio no p\u00e2ncreas, com mais de 10 c\u00e9lulas \/ CGA positivas para IgG4, a fibrose estoriforme e a aus\u00eancia de les\u00f5es granuloc\u00edticas.<\/li>\n<\/ul>\n<p>\u00a0<\/p>\n<ul>\n<li><strong>Pancreatite Autoimune tipo 2<\/strong>: \u00e9 uma doen\u00e7a exclusivamente pancre\u00e1tica, que pode cursar com epis\u00f3dios de Pancreatite Aguda Recorrente, e que tem como caracter\u00edstica o infiltrado granuloc\u00edtico no p\u00e2ncreas e a aus\u00eancia de c\u00e9lulas positivas para IgG4. O diagn\u00f3stico de PAI tipo 2 s\u00f3 pode ser confirmado com a histologia pancre\u00e1tica. Apesar de ser uma doen\u00e7a restrita ao p\u00e2ncreas, tem associa\u00e7\u00e3o com outras condi\u00e7\u00f5es autoimunes, como as Doen\u00e7as Inflamat\u00f3rias Intestinais (especialmente RCUI).<\/li>\n<\/ul>\n<p>\u00a0<\/p>\n<h5><strong>Quadro cl\u00ednico<\/strong><\/h5>\n<ul>\n<li>O <strong>quadro cl\u00ednico<\/strong> t\u00edpico da PAI (em qualquer um dos subtipos) \u00e9 a dor abdominal, icter\u00edcia obstrutiva e eleva\u00e7\u00e3o de enzimas pancre\u00e1ticas e canaliculares no sangue. \u00c9 comum tamb\u00e9m o emagrecimento associado.<\/li>\n<li>Em alguns casos pode-se encontrar massas pancre\u00e1ticas ou biliares, que necessitam diagn\u00f3stico diferencial com neoplasias.<\/li>\n<li>Menos comum \u00e9 a ocorr\u00eancia de pancreatites agudas de repeti\u00e7\u00e3o, especialmente na PAI tipo 2 (4, 5).<\/li>\n<li>A dosagem de IgG4 &gt; 140 mg\/dl, a hipergamaglobulinemia e o FAN + podem ser marcadores secund\u00e1rios da doen\u00e7a sist\u00eamica (PAI tipo 1).<\/li>\n<\/ul>\n<p>\u00a0<\/p>\n<h5><strong>Achados Radiol\u00f3gicos<\/strong><\/h5>\n<p>Associado ao quadro cl\u00ednico, os achados radiol\u00f3gicos podem corroborar o diagn\u00f3stico. Cerca de 85% dos pacientes com PAI t\u00eam altera\u00e7\u00f5es radiol\u00f3gicas compat\u00edveis. O achado mais t\u00edpico \u00e9 o edema e o aumento pancre\u00e1tico (p\u00e2ncreas &#8220;em salsicha&#8221;) e a perda de lobula\u00e7\u00f5es, frequentemente associado a um halo hipoatenuante na tomografia ou na resson\u00e2ncia de abdome com contraste em 15-40% dos casos (6,7).<\/p>\n<p>\u00a0<\/p>\n<p><img fetchpriority=\"high\" decoding=\"async\" class=\"alignnone size-medium wp-image-8084\" src=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem2-300x291.png\" alt=\"\" width=\"300\" height=\"291\" srcset=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem2-300x291.png 300w, https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem2.png 325w\" sizes=\"(max-width: 300px) 100vw, 300px\" \/> <img decoding=\"async\" class=\"alignnone size-medium wp-image-8085\" src=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem1-300x300.png\" alt=\"\" width=\"300\" height=\"300\" srcset=\"https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem1-300x300.png 300w, https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem1-150x150.png 150w, https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem1-70x70.png 70w, https:\/\/endoscopiaterapeutica.net\/pt\/wp-content\/uploads\/2019\/05\/Imagem1.png 318w\" sizes=\"(max-width: 300px) 100vw, 300px\" \/><\/p>\n<p><em>\u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 * Imagens de arquivo pr\u00f3prio<\/em><\/p>\n<p>\u00a0<\/p>\n<p>Menos frequente \u00e9 o acometimento focal, com presen\u00e7a de nodula\u00e7\u00f5es na gl\u00e2ndula, podendo mimetizar neoplasia. Essa forma \u00e9 mais comum na PAI tipo 2 (35-80% de incid\u00eancia) e o essencial \u00e9 fazer o diagn\u00f3stico diferencial com processos mit\u00f3ticos. (7) Nesse contexto, o uso de exames como o Ultrassom Endosc\u00f3pico ou a Colangiopancreatografia\u00a0 Retr\u00f3grada Endosc\u00f3pica podem ser \u00fateis na tentativa de afastar o diagn\u00f3stico de processos neopl\u00e1sicos, j\u00e1 que permitem a obten\u00e7\u00e3o de material para avalia\u00e7\u00e3o histopatol\u00f3gica. (8)<\/p>\n<h5>\u00a0<\/h5>\n<p>\u00a0<\/p>\n<h5><strong>Tratamento<\/strong><\/h5>\n<p>O tratamento inicial \u00e9 com corticoterapia, e ambas as formas da doen\u00e7a tem boa resposta ao curso de corticoide. O tratamento est\u00e1 indicado nos casos que se apresentam com icter\u00edcia obstrutiva e dor abdominal, forma nodular (massas pancre\u00e1ticas ou biliares) , \u00a0quadros simulando colangite esclerosante ou doen\u00e7a extra-pancre\u00e1tica. \u00a0A dose inicial pode ser fixa de 40mg\/dia de prednisona (ou em torno de 0,6 mg\/kg\/dia) pelo per\u00edodo de 4 semanas. Ap\u00f3s esse per\u00edodo \u00e9 recomendada uma reavalia\u00e7\u00e3o cl\u00ednica, laboratorial e de imagem. No caso de melhora, est\u00e1 indicada a redu\u00e7\u00e3o da dose de 5mg por semana at\u00e9 a completa suspens\u00e3o da medica\u00e7\u00e3o. (5, 9)<\/p>\n<p>No paciente que tenha contra-indica\u00e7\u00e3o ao uso de corticoide (especialmente os paciente com <em>diabetes mellitus<\/em> descompensado) o Rituximab (anti CD-20) tamb\u00e9m pode ser usado como agente de primeira linha para indu\u00e7\u00e3o de remiss\u00e3o. \u00a0(9, 10)<\/p>\n<p>Apesar de apresentar uma boa resposta ao tratamento com corticoester\u00f3ides, a taxa de recorr\u00eancia\u00a0 dos sintomas \u00e9 de aproximadamente 30%. Os preditores para reicidiva do quadro s\u00e3o: altos n\u00edveis de IgG4 ao diagn\u00f3stico e acometimento de outros \u00f3rg\u00e3os, especialmente \u00e1rvore biliar. Nesses casos ainda n\u00e3o est\u00e1 claro se \u00e9 necess\u00e1rio um tratamento adjuvante com imunomoduladores (Ciclosporina, Azatioprina, Rituximab) ou se \u00e9 necess\u00e1rio um tempo maior de terapia com corticoester\u00f3ides. (1, 2, 5, 9).<\/p>\n<p>\u00a0<\/p>\n<div class=\"pcrstb-wrap\"><table style=\"height: 456px;\" width=\"762\">\n<tbody>\n<tr>\n<td width=\"183\">\u00a0<\/td>\n<td width=\"183\"><strong>Tipo 1<\/strong><\/td>\n<td width=\"183\"><strong>Tipo 2<\/strong><\/td>\n<\/tr>\n<tr>\n<td width=\"183\"><strong>IgG4<\/strong><\/td>\n<td width=\"183\">Relacionada a IgG4<\/td>\n<td width=\"183\">N\u00e3o relacionada a IgG4<\/td>\n<\/tr>\n<tr>\n<td width=\"183\"><strong>Idade<\/strong><\/td>\n<td width=\"183\">&gt; 60 anos<\/td>\n<td width=\"183\">&gt; 40 anos<\/td>\n<\/tr>\n<tr>\n<td width=\"183\"><strong>Sex<\/strong><\/td>\n<td width=\"183\">Masc &gt; Fem<\/td>\n<td width=\"183\">Masc = Fem<\/td>\n<\/tr>\n<tr>\n<td width=\"183\"><strong>IgG4 s\u00e9rica<\/strong><\/td>\n<td width=\"183\">Elevada<\/td>\n<td width=\"183\">Normal<\/td>\n<\/tr>\n<tr>\n<td width=\"183\"><strong>Histologia<\/strong><\/td>\n<td width=\"183\">C\u00e9lulas IgG4 +<\/td>\n<td width=\"183\">Les\u00f5es epiteliais granuloc\u00edticas<\/td>\n<\/tr>\n<tr>\n<td width=\"183\"><strong>Taxa de remiss\u00e3o<\/strong><\/td>\n<td width=\"183\">Alta<\/td>\n<td width=\"183\">Baixa<\/td>\n<\/tr>\n<tr>\n<td width=\"183\"><strong>Extra-pancre\u00e1tico<\/strong><\/td>\n<td width=\"183\">Doen\u00e7as relacionadas a \u00a0IgG4<\/td>\n<td width=\"183\">DII (30%)<\/td>\n<\/tr>\n<\/tbody>\n<\/table><\/div>\n<p>\u00a0<\/p>\n<h3>P<a href=\"https:\/\/gastropedia.com.br\/gastroenterologia\/insuficiencia-pancreatica-exocrina-um-olhar-alem-do-obvio\/\">ara saber mais sobre este tema e outros relacionados, acesse o site Gastropedia clicando aqu<\/a>i !<\/h3>\n<p>\u00a0<\/p>\n<p><strong>Refer\u00eancias bibliogr\u00e1ficas<\/strong><\/p>\n<ul>\n<li>Mahdani, K. Farrel, J. Management of Autoimmune Pancreatitis. Gastrointest Endoscopy Clin N Am 28, 2018, 493\u2013519<\/li>\n<li>Shimosegawa, T. et al. International Consensus Diagnostic Criteria for Autoimmune Pancreatitis Guidelines of the International Association of Pancreatology. Pancreas 2011; 40: 352-358<\/li>\n<li>Sah, R.P., Chari, S.T. Autoimmune Pancreatitis: An Update on Classification, Diagnosis, Natural History and Management. Curr Gastroenterol Rep, 2012 14:95\u2013105<\/li>\n<li>Hart, P.A. et al. Recent Advances in Autoimmune Pancreatitis. Gastroenterology 2015;149:39\u201351<\/li>\n<li>Nagpal, S.J.S. et al. Autoimmune Pancreatitis. <em>Am J Gastroenterol <\/em>(2018) 113:1301\u20131309<\/li>\n<li>Raina A, Yadav D, Krasinskas AM, et al. Evaluation and management of autoimmune pancreatitis: experience at a large US center. Am J Gastroenterol 2009; 104(9):2295\u2013306.<\/li>\n<li>Sandrasegaran, K. Menias, C.O. Imaging in Autoimmune Pancreatitis and Immunoglobulin G4\u2013Related Disease of the Abdomen. Gastroenterol Clin N Am 47 (2018) 603\u2013619<\/li>\n<li>Fujii-Lau, L.L.. Levy, M.J. The Role of Endoscopic Ultrasound in the Diagnosis of Autoimmune Pancreatitis. Gastrointest Endoscopy Clin N Am, 2017.<\/li>\n<li>Kamisawa, T. et al. Advances in IgG4-related pancreatobiliary diseases. Lancet Gastroenterol Hepatol, 2018; 3: 575\u201385<\/li>\n<li>Okazaki, K. Uchida, K. Current perspectives on autoimmune pancreatitis and IgG4-related disease. Jpn. Acad., Ser. B 94 (2018) 412-427.<\/li>\n<\/ul>\n\n\n<h2 class=\"wp-block-heading\"><\/h2>\n","protected":false},"excerpt":{"rendered":"<p>\u00a0 A Pancreatite Autoimune (PAI) \u00e9 uma das poss\u00edveis causas de pancreatite cr\u00f4nica, que&hellip;<\/p>\n","protected":false},"author":3334,"featured_media":5303,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"","_lmt_disable":"","footnotes":""},"categories":[151],"tags":[],"ano":[145],"tipo":[155,154,159],"volume":[263],"class_list":["post-4628","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-assuntosgerais","ano-145","tipo-cpre","tipo-ecoendoscopia","tipo-outros","volume-volume-i"],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v27.2 (Yoast SEO v27.2) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>Pancreatite Autoimune &#8226; Endoscopia Terapeutica<\/title>\n<meta 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