Enfermedad relacionada con IgG4 en paciente con antecedente de linfoma MALT gástrico: un gran reto diagnóstico y terapéutico / IgG4 related disease in a patient with a history of gastric MALT lymphoma: a great diagnostic and treatment challenge

We describe a case of a 57-year-old woman with a history of gastric MALT lymphoma and interstitial nephritis attributed to chemotherapy. In the study of chronic diarrhea, we found an atrophic pancreas, with elastase deficiency. Autoimmune pancreatitis is suspected. A significant elevation of serum IgG4 was observed. With these data, a review of the renal biopsy performed 10 months earlier was carried out. Immunohistochemistry reveals a significant number of IgG4-producing plasma cells. In the lungs, the patient has nodules, adenopaties and infiltrates. The diagnosis we arrived at is IgG4-related disease. (AU)

Se presenta el caso de una mujer de 57 años con antecedentes de linfoma MALT gástrico y nefritis intersticial atribuida a la quimioterapia. En el estudio de diarrea crónica encontramos un páncreas atrófico, con deficiencia de elastasa. Se sospecha pancreatitis autoinmune. Se comprueba una elevación importante de IgG4 sérica. Con estos datos, se procede a la revisión de la biopsia renal realizada 10 meses antes. La inmunohistoquímica revela un número significativo de células plasmáticas productoras de IgG4. En los pulmones, la paciente tiene nódulos, adenopatías e infiltrados. El diagnóstico al que llegamos es Enfermedad relacionada con IgG4. (AU)

Immunoglobulin G4-Related Disease, A Diagnosis to Take into Account: About a Case

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated and multisystemic condition associated with developing fibroinflammatory lesions in any organ. The diagnosis is made based on the sum of clinical, serological, radiological, and histopathological criteria; however, this is often difficult due to its similarity to neoplasms, infections, or other immune-mediated diseases. Treatment is based on corticosteroids, in a possible combination with immunomodulators. The present case concerns a 59-year-old man with a history of jaundice syndrome and weight loss, admitted for suspected malignant neoplasia of the bile duct. Imaging revealed stricture with dilation of the intrahepatic bile ducts, prominent pancreas, pancreatic duct stricture, and nodular renal lesions. Due to the history of left submandibulectomy two years before the current disease and histology compatible with Küttner's tumor, plus the high IgG4 serum values, the diagnosis of IgG4-RD was established. He started treatment with corticosteroids and was asymptomatic during follow-up.

La enfermedad relacionada con inmunoglobulina G4 (ER-IgG4) es una condición inmunomediada y multisistémica asociada al desarrollo de lesiones fibroinflamatorias en cualquier órgano. El diagnóstico se realiza bajo la suma de criterios clínicos, serológicos, radiológicos e histopatológicos; sin embargo, este suele ser difícil debido a su similitud con neoplasias, infecciones u otras enfermedades inmunomediadas. El tratamiento se basa en corticosteroides, en una posible combinación con inmunomoduladores. El presente caso trata de un varón de 59 años con historia de síndrome ictérico y baja de peso, admitido por sospecha de neoplasia maligna de la vía biliar. Las imágenes revelaron estenosis con dilatación de las vías biliares intrahepáticas, páncreas prominente, estenosis del conducto pancreático y lesiones nodulares renales. Debido al antecedente de submandibulectomía izquierda 2 años antes de la enfermedad actual e histología compatible con tumor de Küttner, más los valores elevados de IgG4 sérico, se estableció el diagnóstico de ER-IgG4. Inició tratamiento con corticosteroides y cursó asintomático durante el seguimiento.

Thoracoabdominal manifestations of immunoglobulin G4-related disease.

Immunoglobulin G4-related disease (IgG4-RD) comprises a group of immune-mediated diseases that, until recently, were considered separate entities. These entities have a similar clinical presentation, serological findings, and pathogenesis, so they are currently considered a single multisystemic disease. The common characteristic is the infiltration of involved tissues by plasma cells and lymphocytes that are positive for IgG4. Three major criteria have been proposed for the diagnosis of IgG4-RD: clinical, laboratory, and histological. The pancreas is the organ that is most affected by IgG4-RD, which can simulate a tumor. In this respect, a series of signs could help us suspect that the pancreatic findings do not reflect a tumor (halo sign, duct-penetrating sign, absence of vascular invasion, etc.). The differential diagnosis is important to avoid unnecessary surgical interventions.

Manifestaciones toracoabdominales de las enfermedades relacionadas con la inmunoglobulina G4 / Thoracoabdominal manifestations of immunoglobulin G4-related disease

Las enfermedades relacionadas con la IgG4 (ER-IgG4) son un conjunto de enfermedades inmunomediadas que, hasta no hace mucho tiempo, se consideraban como entidades individuales. Se ha demostrado la existencia de una clínica, una serología y una patogenia similares, por lo que, actualmente, se considera como una única enfermedad multisistémica. Su característica común es la infiltración de los tejidos afectados por células plasmáticas y linfocitos IgG4 positivos. Se han propuesto 3 criterios mayores para su diagnóstico, que son el clínico, el analítico y el anatomopatológico. El páncreas es el órgano que más se afecta en esta enfermedad, pudiendo simular un proceso tumoral. De ahí que se haya descrito una serie de signos que podrían hacernos sospechar que no se trata de una neoplasia (signo del halo, del conducto penetrante, ausencia de invasión vascular, etc.). El diagnóstico diferencial es importante de cara a evitar cirugías innecesarias (AU)

Immunoglobulin G4-related disease (IgG4-RD) comprises a group of immune-mediated diseases that, until recently, were considered separate entities. These entities have a similar clinical presentation, serological findings, and pathogenesis, so they are currently considered a single multisystemic disease. The common characteristic is the infiltration of involved tissues by plasma cells and lymphocytes that are positive for IgG4. Three major criteria have been proposed for the diagnosis of IgG4-RD: clinical, laboratory, and histological. The pancreas is the organ that is most affected by IgG4-RD, which can simulate a tumor. In this respect, a series of signs could help us suspect that the pancreatic findings do not reflect a tumor (halo sign, duct-penetrating sign, absence of vascular invasion, etc.). The differential diagnosis is important to avoid unnecessary surgical interventions (AU)

IgG4-related disease associated with immune thrombocytopenia: A clinical case report

Autoimmune pancreatitis is a characteristic manifestation of the spectrum of the disease related to IgG4, a rare autoimmune disorder that presents clinically with obstructive jaundice due to the infiltration of plasma cells and fibrosis in the pancreas. There may be other symptoms in case of involvement of other organs, and in very rare cases there is hematological involvement. We present the case of an adult man with signs of cholestasis secondary to type I autoimmune pancreatitis, with involvement of other organs and associated with thrombocytopenia that improved with systemic corticosteroid-based immunosuppressive treatment, after which the patient showed favorable clinical and analytical evolution over time.

La pancreatitis autoimmune es una manifestación característica del espectro de la enfermedad relacionada con IgG4, trastorno raro de tipo autoinmune que se presenta clínicamente con ictericia obstructiva debido a la infiltración de células plasmáticas y fibrosis en el páncreas; puede presentarse con otra sintomatología en caso de afectación de otros órganos y en muy raras ocasiones hay compromiso hematológico. Se presenta el caso de un hombre adulto con signos de colestasis secundaria a una pancreatitis autoinmune tipo i, con compromiso de otros órganos y asociada con trombocitopenia que mejoró con el tratamiento inmunosupresor a base de corticoide sistémico, luego del cual se observó una evolución favorable en cuanto a la clínica y analítica en el transcurso del tiempo.

Autoimmune pancreatitis diagnosis based on immunoglobulin G4 levels: Case report / Diagnóstico de pancreatitis autoinmune basado en niveles de inmunoglobulina G4: reporte de caso

Abstract Introduction: Autoimmune pancreatitis is a rare form of chronic pancreatitis, with an estimated prevalence of 1 case per 100 000 inhabitants in the general population. It is characterized by the presence of painless obstructive jaundice and imaging findings of a diffusely enlarged pancreas and loss of its lobularity. Depending on its features, it can be classified as subtype 1 or 2, and its diagnosis is reached based on imaging, serological, and histological findings, as well as the patient's response to corticosteroid therapy, which in turn is the indicated treatment for this disease. Case presentation: A 62-year-old man was admitted to the emergency department of a quaternary care hospital in Bogotá D.C. due to generalized jaundice associated with choluria and acholia. Upon admission, the patient was assessed by the general surgery and gastroenterology services and diagnosed with autoimmune pancreatitis based on immunoglobulin G4 levels and imaging and laboratory findings compatible with this disease. He was successfully treated with corticosteroids, obtaining satisfactory clinical outcomes and a complete resolution of symptoms. Conclusions: Autoimmune pancreatitis is a disease that should be suspected in patients with symptoms of painless obstructive jaundice, thus avoiding misdiagnoses and providing timely treatment that allows the resolution of symptoms and proper management of the disease.

Resumen Introducción. La pancreatitis autoinmune es una forma de pancreatitis crónica poco frecuente, con una prevalencia estimada de 1 caso por cada 100 000 habitantes en la población general. Se caracteriza por la presencia de ictericia obstructiva indolora y hallazgos imagenológicos de un páncreas aumentado de tamaño con forma difusa y pérdida de su lobularidad. Se clasifica en subtipo 1 o 2, dependiendo de sus características, y el diagnóstico se realiza de acuerdo con los hallazgos imagenológicos, serológicos, histológicos y la respuesta a la corticoterapia, que a su vez es el tratamiento indicado para esta enfermedad. Presentación del caso. Hombre de 62 años quien ingresó al servicio de urgencias de un hospital de cuarto nivel de atención en Bogotá D.C., Colombia, por cuadro clínico de ictericia generalizada acompañada de coluria y episodios de acolia. Fue valorado por el servicio de cirugía general en conjunto con gastroenterologia y diagnosticado con pancreatitis autoinmune basándose en los niveles de inmunoglobulina G4 y los hallazgos imagenológicos y de laboratorio compatibles con esta enfermedad. El paciente fue tratado de manera exitosa con terapia corticoide, logrando resultados clínicos satisfactorios y resolución de la sintomatologia. Conclusiones. La pancreatitis autoinmune es una enfermedad que debe sospecharse en pacientes con sintomatologia de ictericia obstructiva indolora, evitando de esta forma diagnósticos erróneos y brindando un tratamiento oportuno que permita la resolución de los síntomas y un manejo adecuado de la enfermedad.

IgG4 related disease. / Enfermedad relacionada con IgG4.

IgG4-related disease (ER-IgG4) is a recognised systemic disease. It was described after patients diagnosed with autoimmune pancreatitis showed signs of extra-pancreatic disease. The clinical manifestation of these patients is subacute and is manifested by the appearance of pseudotumoral lesions, or inflammatory or fibrous tumours. Sometimes it can be serious as in the case of patients with cholangitis or large vessel vasculitis. Diagnostic criteria include, among others, serum IgG4 elevation and/or histological parameters. The 18F-FDG-PET/CT is useful in the initial diagnosis, biopsy guidance as well as in the assessment of response to therapy. It usually responds to steroid therapy.

Differential diagnosis between pancreatic involvement in IgG4-related disease and pancreatic cancer. / Diagnóstico diferencial entre compromiso pancreático en enfermedad relacionada con IgG4 y cáncer de páncreas.

IgG4-related disease is a systemic disorder characterised by diffuse or tumoural inflammatory lesions. It can mimic pancreatic cancer, leading to errors in diagnosis and treatment increasing rates of morbidity and mortality in patients. The aim of this review is to take a differential diagnostic approach to these two entities using epidemiology, clinical and laboratory findings, imaging and histopathology.

Review of IgG4-related disease. / Revisión de la enfermedad relacionada con la IgG4.

IgG4-related disease is a fibrous-inflammatory process related to immunomodulation. The most commonly affected organs are: the pancreas, bile duct, major salivary glands, lacrimal glands, retroperitoneum and lymphatic ducts. In recent decades, this disease has been recognised as a systemic disorder that includes many single organ disorders, previously unrelated and known as independent entities. The common characteristics shared by the different entities that make up the IgG4-related disease are: raised serum IgG4 levels, alterations in the imaging tests with neoplastic-like swelling of the affected organs, specific histopathological characteristics and in immunostaining, as well as good response to treatment with glucocorticoids. In this work, we will review this pathology with a special emphasis on the characteristics of autoimmune pancreatitis, sclerosing cholangitis related to IgG4 and the involvement of the retroperitoneum, mesenterium and the digestive tract.

Manifestaciones gastrointestinales en Lupus Eritematoso Sistémico: un espectro diferente / Gastrointestinal manifestations in Lupus Erythematosus Systemic: a different spectrum

Presentamos el caso de escolar femenina, que debuta con colecistitis y pancreatitis aguda como manifestación inicial del lupus eritematoso sistémico. Consulta por ebre de origen por determinar e hiporexia. Por la persistencia de la fiebre, pérdida de peso y manifestaciones sistémicas se sospecha de una Enfermedad Autoinmune, con rmando el diagnóstico de LES con ANA y antiDNA positivos

We present the case of a school-aged female, who presents with acute cholecystitis and acute pancreatitis as the initial manifestation of systemic lupus erythematosus. She consults with fever of unknown origin and hyporexia. Due to persistent fever, weight loss and systemic manifestations, Autoimmune Disease is suspected, confirming the diagnosis of SLE with positive ANA and anti-DNA with which the diagnosis of SLE was made

Enfermedad por IgG4 multisistémica: a propósito de un caso: revisión de la literatura / Multisystemic IgG4 disease: a case report: literature review

We report a 63-year-old male patient who consulted for a 4-month history of xerophthalmia, xerostomia and cervicalgia, associated with jaundice, choluria, low weight and night sweats. Exams show an obstructive pattern and abdominal ultrasound describes a possible mass in the pancreatic head with secondary dilation of the bile duct. Colangio-MRI (magnetic resonance imaging) confirms a focal pancreatic head lesion that determines intrapancreatic bile duct stenosis, proximal dilation of the bile duct and stenosis of the main pancreatic duct. Pancreatic neoplasia versus autoimmune pancreatitis (PAI) is proposed, complementing a study with a count of IgG subclasses where IgG4 stands out at 1050 mg/dl (normal range: 3.9-86) and computed tomography (CT) of the thorax, abdomen and pelvis, that in addition to the biliary-pancreatic alterations, shows thickening of the vesicular wall, multiple bilateral lesions in the renal parenchyma and peri-aortic soft tissue. Salivary gland biopsy reports lymphoplasmacytic infiltrate characteristic of IgG4 disease. A disease related to IgG4 (ER-IgG4) is diagnosed with pancreatic, renal, biliary, vascular, lymphatic, salivary and lacrimal glands involvement. It is treated with corticoidal therapy, evolving favorably with resolution of the symptomatology and regression of imaging alterations.

Se reporta caso de paciente de sexo masculino de 63 años que consulta por cuadro de 4 meses de xeroftalmia, xerostomía y cervicalgia, asociado a ictericia, coluria, baja de peso y sudoración nocturna. En exámenes destaca pruebas hepáticas con un patrón obstructivo y ecografía abdominal que muestra una posible masa en la cabeza pancreática con dilatación secundaria de la vía biliar. Colangio-resonancia (resonancia magnética-RM) confirma una lesión focal en la cabeza pancreática que determina estenosis del colédoco intrapancreático, dilatación proximal de la vía biliar y estenosis del conducto pancreático principal. Se plantea neoplasia de páncreas versus pancreatitis autoinmune (PAI), complementando estudio con recuento de subclases de IgG donde destaca IgG4 de 1.050 mg/dl (rango normal: 3,9-86) y tomografía computada (TC) de tórax, abdomen y pelvis, que además de las alteraciones bilio-pancreáticas, demuestra engrosamiento de la pared vesicular, lesiones múltiples bilaterales en el parénquima renal y tejido de partes blandas peri-aórtico. Biopsia de las glándulas salivales informa sialoadenitis crónica e inespecífica. Se diagnostica una enfermedad relacionada a IgG4 (ER-IgG4) con compromiso pancreático, renal, biliar, vascular, linfático, de glándulas salivales y lagrimales. Se trata con terapia corticoidal, evolucionando favorablemente con resolución de la sintomatología y regresión de las alteraciones imagenológicas.

Espectro clínico de la enfermedad relacionada con IgG4 en Colombia / Clinical spectrum of IgG4-related disease in Colombia

RESUMEN La enfermedad relacionada con IgG4 es una condición inflamatoria sistémica, caracterizada por la infiltración de diversos órganos por complejos formados por células plasmáticas IgG4 positivas, asociadas con niveles elevados de IgG4 en el suero. El diagnóstico de esta enfermedad es complejo y los hallazgos clínicos no son patognomónicos. En esta serie de casos, describimos un amplio espectro clínico en 4 pacientes, en quienes, después de descartar otros diagnósticos, se confirmó la enfermedad relacionada con IgG4. A pesar de que esta enfermedad no es común, se debe considerar entre los diagnósticos diferenciales de enfermedades con afectación de múltiples órganos. Este reporte de pacientes con enfermedad relacionada con IgG4, en Colombia, resalta un amplio espectro de presentaciones clínicas, incluyendo estenosis subglótica, pancreatitis autoinmune, fibrosis retroperitoneal y compromiso sistémico.

ABSTRACT IgG4-related disease is an inflammatory systemic condition noted by the infiltration of different organs by IgG4-bearing plasma cells, as well as elevated serum IgG4 levels. Diagnosis of this condition is complex, and clinical findings are not particularly indicative. In this case series, a description is presented on 4 patients with a wide spectrum of clinical manifestations, in whom, after ruling out different options, a diagnosis of IgG4 related disease was confirmed. Despite this disease not being common, it should be considered among the options whenever multiple organs are affected. This report of patients with IgG4-related disease in Colombia highlights a wide spectrum of clinical presentations, including subglottic stenosis, autoimmune pancreatitis, retroperitoneal fibrosis, and systemic compromise.

IgG4-related disease: A concise review of the current literature. / Enfermedad relacionada con IgG4: revisión concisa de la literatura.

IgG4-related disease is the term used to refer to a condition characterized by a lymphoplasmacytic infiltrate, fibrosis and an increased number of IgG4+ cells present in tissue, in most cases, with an elevated serum IgG4 level. This disease frequently affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. Its etiology and the exact role of the different inflammatory cells in the damage to the target organ is still unclear. As yet, there is no international consensus about diagnostic criteria for the disease, but there are important advances in its treatment and in the quest to achieve remission. We include a review of the history, possible pathogenesis, clinical manifestations, diagnostic approach and available therapeutic approaches.

Latest advances in chronic pancreatitis. / Últimos avances en pancreatitis crónica.

This article summarizes some of the recent and clinically relevant advances in chronic pancreatitis. These advances mainly concern the definition of the disease, the etiological diagnosis of idiopathic disease, the correlation between fibrosis degree and pancreatic secretion in the early stages of chronic pancreatitis, the treatment of the disease and of pain, the clinical relevance of pancreatic exocrine insufficiency, and the diagnosis of autoimmune pancreatitis. A new mechanistic definition of chronic pancreatitis has been proposed. Genetic testing is mainly of help in patients with relapsing idiopathic pancreatitis. A significant correlation has been shown between the degree of pancreatic fibrosis as evaluated by elastography and pancreatic secretion of bicarbonate. New data supports the efficacy of antioxidants and simvastatin for the therapy of chronic pancreatitis. The pancreatoscopy-guided intraductal lithotripsy is an effective alternative to extracorporeal shock wave lithotripsy in patients with chronic calcifying pancreatitis. The presence of pancreatic exocrine insufficiency in patients with chronic pancreatitis is associated with a significant risk of cardiovascular events. Fine needle biopsy and contrast enhanced harmonic endoscopic ultrasonography are of help for the diagnosis of autoimmune pancreatitis and its differential diagnosis with pancreatic cancer.

[Latest advances in chronic pancreatitis]. / Últimos avances en pancreatitis crónica.

This article summarizes some of the recent and clinically relevant advances in chronic pancreatitis. These advances mainly concern the early diagnosis of the disease, the treatment of symptoms and complications, mainly pain and pancreatic exocrine insufficiency, and the diagnosis and therapy of autoimmune pancreatitis. The multimodal dynamic endoscopic ultrasound-guided secretin-stimulated evaluation of the pancreas provides relevant morphological and functional information for the diagnosis of chronic pancreatitis at early stages. Extracorporeal shock wave lithotripsy in patients with calcifying pancreatitis and endoscopic pancreatic stent placement are effective alternatives for pain therapy in patients with chronic pancreatitis. Presence of pancreatic exocrine insufficiency in patients with chronic pancreatitis is associated with a significantly increase of mortality rate. Despite that, pancreatic enzyme replacement therapy is not prescribed in the majority of patients with pancreatic exocrine insufficiency, or it is prescribed at a low dose. The newly developed and commercialized needles for endoscopic ultrasound-guided pancreatic biopsy are effective in retrieving appropriate tissue samples for the histological diagnosis of autoimmune pancreatitis. Maintenance therapy with azathioprine is effective and safe to prevent relapses in patients with autoimmune pancreatitis.

[Autoimmune pancreatitis]. / Pancreatitis autoinmune.

Autoimmune pancreatitis is a benign fibroinflammatory disease of the pancreas of probable autoimmune origin, which includes 2 different phenotypes: type 1 (lymphoplasmacytic sclerosing pancreatitis) and type 2 (idiopathic duct-centric pancreatitis). Its clinical presentation as obstructive jaundice in patients with a pancreatic mass is common and therefore it must be included in the differential diagnosis of pancreatic neoplasia. Many diagnostic criteria have been described throughout history. The most famous are the HISORT criteria of the Mayo Clinic and the international consensus criteria of 2011. One of the main features of autoimmune pancreatitis is its dramatic response to steroid therapy, without the need for surgical treatment. Knowledge of this disease can dramatically change the management of patients with obstructive jaundice, who would otherwise be subjected to a pancreaticoduodenectomy.

[IgG4-related disease]. / Enfermedad relacionada con IgG4.

IgG4-related disease is a recently described clinicopathological entity showing a wide spectrum of clinical manifestations that share a common pathology. Its most characteristic feature is the formation of inflammatory tumors in different organs, which makes differentiation mainly with neoplastic diseases fundamental. The inflammatory process is typically comprised of IgG4 lymphoplasmacytic cells. The pathophysiological role of the immunoglobulin is not clear. The treatment of choice is corticosteroids. This article aims to summarize the main features of the disease.

Pancreatitis aguda en paciente con lupus eritematoso sistémico / Acute pancreatitis in a patient with systemic lupus erythematosus

Se presenta caso de mujer joven portadora de lupus eritematoso sistémico que consulta por síntomas gastrointestinales y fiebre. Por laboratorio y tomografía abdominal se llega a diagnóstico de pancreatitis aguda que mejora con corticoides.

Report case of a young women carrier of systemic lupus erythematosus who comes to consult with gastrointestinal symptoms and fever. The diagnosis of Acute Pancreatitis is reached with laboratory exams and abdominal CT. Corticosteroids have afficacy for the treatment.

[Latest advances in chronic pancreatitis]. / Últimos avances en pancreatitis crónica.

This article summarizes some of the recent and clinically relevant advances in chronic pancreatitis. These advances mainly concern the early diagnosis of the disease, the prediction of the fibrosis degree of the gland, the evaluation of patients with asymptomatic hyperenzimemia, the medical and surgical treatment of abdominal pain and the knowledge of the natural history of the autoimmune pancreatitis. In patients with indetermined EUS findings of chronic pancreatitis, a new endoscopic ultrasound examination in the follow-up is of help to confirm or to exclude the disease. Smoking, number of relapses, results of pancreatic function tests and EUS findings allow predicting the degree of pancreatic fibrosis in patients with chronic pancreatitis. Antioxidant therapy has shown to be effective in reducing pain secondary to chronic pancreatitis, although the type and optimal dose of antioxidants remains to be elucidated. Development of intestinal bacterial overgrowth is frequent in patients with chronic pancreatitis, but its impact on symptoms is unknown and deserves further investigations. Finally, autoimmune pancreatitis relapses in about half of the patients with either type 1 or type 2 disease; relapses frequently occur within the first two years of follow-up.

Autoimmune pancreatitis: a surgical dilemma.

Autoimmune pancreatitis (AIP) is defined as a particular form of pancreatitis that often manifests as obstructive jaundice associated with a pancreatic mass or an obstructive bile duct lesion, and that has an excellent response to corticosteroid treatment. The prevalence of AIP worldwide is unknown, and it is considered as a rare entity. The clinical and radiological presentation of AIP can mimic bilio-pancreatic cancer, presenting difficulties for diagnosis and obliging the surgeon to balance decision-making between the potential risk presented by the misdiagnosis of a deadly disease against the desire to avoid unnecessary major surgery for a disease that responds effectively to corticosteroid treatment. In this review we detail the current and critical points for the diagnosis, classification and treatment for AIP, with a special emphasis on surgical series and the methods to differentiate between this pathology and bilio-pancreatic cancer.