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Pancreatite autoimune : o que sabemos actualmente
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Background: A doença relacionada com a imunoglobulina G4 (DR-IgG4) é uma entidade sistémica caracterizada por elevação da IgG4 sérica, hipertrofia dos orgãos afectados com infiltração linfoplasmocítica maciça com plasmócitos IgG4-positivos e fibrose tecidular. A pancreatite autoimune (PAI) é uma forma de pancreatite com provável etiologia autoimune que pode apresentar envolvimento difuso ou focal do pâncreas. A PAI tipo 1 é a manifestação pancreática da DR-IgG4 e a Tipo 2 uma doença específica do pâncreas. Na forma focal a PAI partilha características com o adenocarcinoma do pâncreas.
Objectivos: Realizar uma revisão do conhecimento actual da PAI quanto à patogénese, epidemiologia, características clínicas, histológicas, imagiológicas, manifestações extrapancreáticas da DR-IgG4 e tratamento. Focam-se as descobertas recentes quanto ao diagnóstico e diagnóstico diferencial com adenocarcinoma do pâncreas.
Métodos: Pesquisa na base de dados da Pubmed usando as palavras DR-IgG4 e PAI. Seleccionei apenas os artigos entre 2006-2015.
Conclusões: Apesar da intensa investigação, o diagnóstico diferencial entre PAI e adenocarcinoma do pâncreas ainda é desafiador para os clínicos. A sua correcta diferenciação pode evitar cirúrgia desnecessária em doentes com PAI, pois estes podem ser tratados com sucesso com corticoesteroides e rituximab. Se não for possível excluir com segurança o adenocarcinoma do pâncreas a cirurgia é mandatória.
Background: IgG4-Related Disease (IgG4-RD) is a systemic disease characterized by elevation of serum IgG4, enlargement of the affected organs with lymphoplasmacitic infiltration with IgG4-positive plasmocytes and tissue fibrosis. Autoimmune pancreatitis (AIP) is a form of pancreatitis with probable autoimmune etiology that can present as focal or diffuse form of involvement. Type 1 AIP is the pancreatic manifestation of IgG4-RD whereas Type 2 is a pancreas-specific disorder. The AIP focal form shares features with pancreatic cancer. Objectives: We intend to make a brief review on the current knowledge about AIP regarding patogenesis, epidemiology, histologic, clinical and imagiological features, laboratory findings, extrapancreatic involvement and treatment. We will focus the scientific breakthroughs in the last years concerning the diagnosis and differential diagnosis with pancreatic adenocarcinoma. Methods: Selective literature research in Pubmed database regarding IgG4-RD and AIP. Only those articles between 2006 and 2015 were selected. Conclusion: Although the intensive investigation in the last years, the differential diagnosis between AIP and pancreatic adenocarcinoma is still a challenge for clinicians. The correct differentiation between them can prevent unnecessary surgery in AIP patients since these can be successfully treated conservatively with steroids and rituximab. If pancreatic adenocarcinoma cannot be excluded with safety surgery is mandatory.
Background: IgG4-Related Disease (IgG4-RD) is a systemic disease characterized by elevation of serum IgG4, enlargement of the affected organs with lymphoplasmacitic infiltration with IgG4-positive plasmocytes and tissue fibrosis. Autoimmune pancreatitis (AIP) is a form of pancreatitis with probable autoimmune etiology that can present as focal or diffuse form of involvement. Type 1 AIP is the pancreatic manifestation of IgG4-RD whereas Type 2 is a pancreas-specific disorder. The AIP focal form shares features with pancreatic cancer. Objectives: We intend to make a brief review on the current knowledge about AIP regarding patogenesis, epidemiology, histologic, clinical and imagiological features, laboratory findings, extrapancreatic involvement and treatment. We will focus the scientific breakthroughs in the last years concerning the diagnosis and differential diagnosis with pancreatic adenocarcinoma. Methods: Selective literature research in Pubmed database regarding IgG4-RD and AIP. Only those articles between 2006 and 2015 were selected. Conclusion: Although the intensive investigation in the last years, the differential diagnosis between AIP and pancreatic adenocarcinoma is still a challenge for clinicians. The correct differentiation between them can prevent unnecessary surgery in AIP patients since these can be successfully treated conservatively with steroids and rituximab. If pancreatic adenocarcinoma cannot be excluded with safety surgery is mandatory.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2015
Palavras-chave
IgG4-related disorder Immnoglobulin G4-related systemic disease Autoimmune pancreatitis Pancreatic imaging Sclerosing cholangitis Pancreatic adenocarcinoma