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.

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.

The role of endoscopic ultrasonogaphy in differentiating between autoimmune pancreatitis and pancreatic cancer / 中华消化内镜杂志

Objective:To investigate the role of endoscopic ultrasonography (EUS) in differentiating between autoimmune pancreatitis (AIP) and pancreatic cancer (PC).Methods:Data of 133 patients with AIP and 113 patients with PC who underwent EUS because of obstructive jaundice at Peking Union Medical College Hospital from January 2013 to December 2018 were retrospectively analyzed in the study, and were randomly divided into either a derivation sample or a validation sample using 1∶1 allocation according to the random number. In the derivation sample, 10 EUS characteristics were used to construct a prediction model to distinguish between AIP and PC, in which predictors were identified by multivariate stepwise logistic regression analysis and predictive efficacy was evaluated by receiver operating characteristics (ROC) curve analysis. The predictive efficacy was assessed in the validation sample. In view of the subjectivity in the judgment of diffuse/focal hypoechogenicity, 2 prediction models were designed in order to avoid bias.Results:By multivariate stepwise logistic regression analysis, diffuse hypoechogenicity ( OR=591.0, 95% CI: 98.8->999.9, P<0.001) and vessel involvement ( OR=11.9, 95% CI: 1.4-260.2, P=0.023) were identified as statistically significant predictors for distinguishing AIP from PC. EUS characteristics excluding diffuse/focal hypoechogenicity were stepped by logistic regression, which showed that hyperechoic foci/strands ( OR=177.3, 95% CI: 18.7->999.9, P<0.001), pancreatic duct dilation ( OR=60.5, 95% CI: 6.2->999.9, P=0.004), bile duct wall thickening ( OR=35.4, 95% CI: 3.7->999.9, P=0.009), lymphadenopathy ( OR=16.8, 95% CI: 1.7-475.2, P=0.038) and vessel involvement ( OR=22.7, 95% CI: 2.0-725.7, P=0.028) were statistically significant predictors to distinguish the two diseases. Both prediction models were built in the derivation sample, with area under the ROC curve of 0.995 and 0.979 respectively. In the validation sample, sensitivity, specificity, accuracy, positive predictive value and negative predictive value of both prediction models were all >90% by using the optimal cutoff value. Even for discrimination between focal AIP and PC, sensitivity and accuracy of both models were >90%, and specificity, positive predictive value and negative predictive value were all >85%. Conclusion:The 2 prediction models have good differential predictive value, and EUS is a useful tool to differentiate between AIP and PC.

Enfermedad relacionada con IgG4: reporte de un caso y revisión de la literatura / IgG4-related disease: a case report and literature review

Immunoglobulin G4 (IgG4-RD) -related disease is a regional or systemic fibroinflammatory disease of unknown etiology. It has a characteristic histopathological appearance of dense lymphoplasmacytic infiltrates with abundant IgG4 positive plasma cells, storiform fibrosis and obliterative phlebitis with the appearance of inflammatory swelling or swollen lesions. This entity frequently affects the pancreas, salivary glands, and lymph nodes, but it can compromise almost any structure in the human anatomy. This new disease entity includes a wide variety of diseases such as Mikulicz disease, autoimmune pancreatitis, Riedel's thyroiditis, interstitial nephritis, and retroperitoneal fibrosis. Glucocorticoid therapy can resolve clinical and pathologic abnormalities and impaired organ function. IgG4-RD was internationally recognized in 2011, and new evidence has accumulated on its pathogenesis, clinical characteristics, and treatment. However, much is still unknown about the behavior of IgG4 in vivo, the participation of this molecule in disease, and whether its role in IgG4-related disease is primary or secondary. The text below is based on a brief review of the most recent literature on this entity in relation to a clinical case.

One case of diagnosis and treatment of pancreatic neuroendocrine neoplasms with suspected autoimmune pancreatitis / 中华内分泌外科杂志

Because of low incidence, atypical clinical symptom, pancreatic neuroendocrine tumor (pNENs) and autoimmune pancreatitis (AIP) alway have suspected diagnosis and misdiagnosis. This paper aims to improve the diagnosis and treatment of two diseases by a case of pancreatic neuroendocrine tumor with suspected autoimmune pancreatitis.

Clinical features and prognosis of autoimmune pancreatitis alone or with IgG4-related sclerosing cholangitis / 临床肝胆病杂志

ObjectiveTo investigate the clinical features, diagnosis, treatment, and prognosis of autoimmune pancreatitis (AIP) alone versus AIP with IgG4 sclerosing cholangitis (IgG4-SC). MethodsA retrospective analysis was performed for the clinical data of 40 patients with type 1 AIP who were admitted to The First Affiliated Hospital of Zhengzhou University from June 2015 to January 2020, among whom 29 patients had AIP alone and 11 had AIP with IgG4-SC. The two groups were compared in terms of clinical manifestations, laboratory examination, imaging findings, treatment, and prognosis. The t-test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups; the Fisher’s exact test was used for comparison of categorical data between two groups. The Kaplan-Meier method was used to calculate recurrence rate and plot recurrence curve, and the log-rank test was used for univariate analysis. ResultsCompared with the AIP group, the AIP+IgG4-SC group had significantly higher number of affected organs ［3.0(3.0-4.0) vs 3.0(1.5-3.5), Z=-2.172, P=0035］ and response index before treatment ［12.0(12.0-15.0) vs 12.0(9.0-13.5), Z=-2.157, P=0.032］. The AIP+IgG4-SC group had a significantly higher median serum IgG level than the AIP group ［21.0(15.8-23.7) g/L vs 14.8(13.3-15.7) g/L, Z=-2.711, P=0.004］. During the median follow-up time of 15.8 (6.5-31.3) months, the AIP+IgG4-SC group had a significantly higher recurrence rate than the AIP group (χ2=8.155, P=0.004). ConclusionPatients with AIP and IgG4-SC tend to have higher serum IgG4 level, number of affected organs, and recurrence rate than those with AIP alone. Early identification, diagnosis, and treatment can reduce the recurrence rate of AIP.

Pancreatic Manifestations in Inflammatory Bowel Disease: A Review Article / 胃肠病学

Some of the patients with inflammatory bowel disease (IBD) can develop symptoms originated from extragastrointestinal organs and systems, and are called extraintestinal manifestations (EIMs). EIMs may be associated with IBD-related injuries or being drug-induced. These manifestations may occur before, after or simultaneously with the diagnosis of IBD. EIMs may detrimentally impact patient's functional status and quality of life. This article reviewed the pancreatic manifestations in patients with IBD and its underlying pathophysiology. EIMs may complicate the course of IBD. The clinicians must pay real attention to EIMs for facilitating the early diagnosis and treatment.

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.

The value of CT texture analysis in differentiating autoimmune pancreatitis from pancreatic ductal adenocarcinoma / 实用放射学杂志

Objective To investigate the feasibility of the CT texture analysis (CTTA)in differentiating autoimmune pancreatitis (AIP)from pancreatic ductal adenocarcinoma (PDAC).Methods 25 patients with AIP and 31 patients with PDAC who confirmed by pathological or clinical underwent pretreatment three-phase contrast-enhanced CT were enrolled.Histogram parameters (mean CT values,median CT values,25 th,75 th percentile CT values,skewness,kurtosis,entropy and uniformity)were derived from CT images through texture analysis.The differences of histogram parameters between AIP and PDAC groups were compared.ROC and AUC were used to evaluate the diagnostic efficacy of histogram parameters in differentiating AIP from PDAC.Results The values for mean CT values,median CT values,25 th,75 th percentile CT values and uniformity of AIP were significantly higher than those of PDAC group,while the values for entropy of AIP were significantly lower than those of PDAC group in arterial phase,portal phase,and delay phases (all P＜0.05). There were no significant differences in kurtosis and skewness between AIP and PDAC groups (all P＞0.05).The uniformity in portal phase achieved the optimal diagnostic accuracy in differentiating AIP from PDAC (AUC＝0.973 ),the cutoff value was 0.797,the corresponding sensitivity and specificity were 92% and 9 6.8%,respectively.Conclusion CTTA can be used as a quantitative analysis method for differential diagnosis between AIP and PDAC,providing a reference for clinicians to select therapeutic schedules.

Diagnosis and treatment of autoimmune pancreatitis and the value of surgery / 中国实用外科杂志

OBJECTIVE: To summarize the experience of diagnosis and treatment of autoimmune pancreatitis(AIP)and to investigate the value of surgery in the management of AIP.METHODS: The clinical data of 17 patients with AIP who were admitted to Fujian Medical University Union Hospital between January 2013 and June 2017 were analyzed retrospectively. Observed indexes included clinical manifestations,blood test results,imaging findings,diagnosis and treatment and follow-up status. RESULTS:(1)Clinical manifestations:of the 17 patients,7 demonstrated obstructive jaundice,8 had upper abdominal pain,1 had recurrent diarrhea and 1 was found a pancreatic tumor by health checkup. Extrapancreatic organ involvement was detected in 11 of the 17 patients.(2)Blood test results:13 patients underwent serum IgG4 detection and increased expression of IgG4 was found in 13 patients.Increased expression of serum CA199 was detected in 4 patients. Elevated level of serum amylase was found in 3 patients.(3)Imaging findings:all the 17 patients underwent enhanced computed tomography(CT)scan of pancreas.Diffused enlargement of pancreas was observed in 12 patients and localized pancreatic enlargement was observed in 5 patients.(4)Diagnosis and treatment:All of the 17 patients were diagnosed as type 1 AIP. Three of the 17 patients underwent operation due to suspicion of pancreatic cancer and were diagnosed as AIP based on postoperative pathological examinations, including 2 pancreaticoduodenctomy and 1 distal pancreatectomy with splenectomy.Fourteen patients received initial steroid treatment. After steroid treatment,11 patients achieved remission, 1 underwent distal pancreatectomy with splenectomy due to continuous elevation of serum CA199,1 underwent pancreaticoduodenctomy because of a continuous enlarged pancreatic pseudocyst in the head of pancreas with obstructive jaundice,and 1 underwent cystjejunostomy due to a pancreatic pseudocyst in the body of pancreas with abdominal pain.(5)Follow-up status:All of the 17 patients were followed up for an average time of 29 months(range,12-66 months). Three patients who initially underwent pancreatectomy achieved remission after operation and no recurrence was observed. Of the 14 patients who received initial steroid treatment,11 got remission and the other three patients received maintenance steroid treatment for 3 months after operation and no recurrence was observed. CONCLUSION: The diagnosis of AIP should be based on the clinical manifestations,blood test results,imaging findings and pathological examinations. Steroid is the most critical therapy for AIP,but surgery is still important for the management of AIP,especially for localized AIP which is difficultly distinguished from pancreatic cancer and AIP with pancreatic pseudocyst which is refractory to steroid treatment.

Autoimmune pancreatitis with spontaneous remission on 18F-fluorodeoxyglucose positron emission tomography/computed tomography / Journal of Rural Medicine

A 79-year-old man with elevated blood glucose was started on insulin therapy. IgG4 was as high as 1,830 mg/dL, and 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) confirmed diffuse pancreatic enlargement and accumulation of FDG. Based on the above, autoimmune pancreatitis (AIP) was diagnosed, but steroid treatment was not performed. IgG4 later declined, and FDG accumulation in the pancreas disappeared on FDG-PET/CT at the age of 83 years. AIP was thought to have gradually remitted spontaneously over time. FDG-PET/CT is useful for evaluating AIP activity.

Coexisting Mucinous Cystic Neoplasm of the Pancreas and Type 1 Autoimmune Pancreatitis

Type 1 autoimmune pancreatitis (AIP1) is an IgG4-related systemic disease that mimics tumors. We report a rare case of AIP1 accompanied by mucinous cystic neoplasm (MCN). A pancreatic lesion was incidentally detected in a woman in her 60s. After 6 years of follow-up, the lesion abruptly increased in size. Computed tomography showed a 3.5 cm unilocular cyst in the tail of the pancreas and distal pancreatectomy was performed. On microscopic examination, the cyst was lined by mucinous and non-mucinous epithelial cells with mild cytologic atypia. The surrounding stroma comprised ovarian-type spindle cells with progesterone receptor positivity. The pericystic pancreas exhibited multifocal lymphoid follicles, lymphoplasmacytic infiltrations, obliterative phlebitis, and storiform fibrosis. IgG4-positive plasma cell infiltration (215 cells high-power field) and the IgG4/IgG ratio (57%) were increased. Cases of MCN coexisting with AIP1 are extremely rare; only two such cases have been reported in the English-language literature. This third case featured low-grade MCN with AIP1.

Differentiation of autoimmune pancreatitis and pancreatic ductal adenocarcinoma based on multi-modality texture features in F-FDG PET/CT / 生物医学工程学杂志

Autoimmune pancreatitis (AIP) is a unique subtype of chronic pancreatitis, which shares many clinical presentations with pancreatic ductal adenocarcinoma (PDA). The misdiagnosis of AIP often leads to unnecessary pancreatic resection. F-FDG positron emission tomography/ computed tomography (PET/CT) could provide comprehensive information on the morphology, density, and functional metabolism of the pancreas at the same time. It has been proved to be a promising modality for noninvasive differentiation between AIP and PDA. However, there is a lack of clinical analysis of PET/CT image texture features. Difficulty still remains in differentiating AIP and PDA based on commonly used diagnostic methods. Therefore, this paper studied the differentiation of AIP and PDA based on multi-modality texture features. We utilized multiple feature extraction algorithms to extract the texture features from CT and PET images at first. Then, the Fisher criterion and sequence forward floating selection algorithm (SFFS) combined with support vector machine (SVM) was employed to select the optimal multi-modality feature subset. Finally, the SVM classifier was used to differentiate AIP from PDA. The results prove that texture analysis of lesions helps to achieve accurate differentiation of AIP and PDA.

Type 2 Autoimmune Pancreatitis (Idiopathic Duct-Centric Pancreatitis) Highlighting Patients Presenting as Clinical Acute Pancreatitis: A Single-Center Experience

BACKGROUND/AIMS: Type 2 autoimmune pancreatitis (AIP) has been considered extremely rare in East Asia. This study aimed to clarify the prevalence, clinical characteristics and radiological findings of type 2 AIP highlighting patients presenting as acute pancreatitis in a single center. METHODS: Type 2 AIP patients were classified according to International Consensus Diagnostic Criteria. Radiological findings were compared between type 2 AIP presenting as acute pancreatitis and gallstone pancreatitis. RESULTS: Among 244 patients with AIP, 27 (11.1%) had type 2 AIP (definite, 15 [55.5%] and probable 12 [44.5%]). The median age of patients with type 2 AIP was 29 years (interquartile range, 20 to 39 years). Acute pancreatitis was the most common initial presentation (n=17, 63%) while obstructive jaundice was present in only one patient. Ulcerative colitis (UC) was associated with type 2 AIP in 44.4% (12/27) of patients. Radiological pancreatic imaging such as delayed enhancement of diffusely enlarged pancreas, homogeneous enhancement of focal enlargement/mass, absent/minimal peripancreatic fat infiltration or fluid collection, and multifocal main pancreatic duct narrowings were helpful for differentiating type 2 AIP from gallstone pancreatitis. During follow-up (median, 32.3 months), two patients (2/25, 8%) experienced relapse. CONCLUSIONS: In South Korea, type 2 AIP is not as rare as previously thought. Overall, the clinical profile of type 2 AIP was similar to that of Western countries. Type 2 AIP should be considered in young UC patients with acute pancreatitis of uncertain etiology.

Clinical characteristics of 27 patients with type 1 autoimmune pancreatitis / 中华肝胆外科杂志

Objective To analyze the clinical characteristics of type 1 autoimmune pancreatitis (AIP) in patients.Methods The clinical data of 27 patients with type 1 AIP treated at Fujian Provincial Hospital from January 2012 to October 2018 were retrospectively analyzed.Results There are 25 males (92.6％) and 2 females (7.4％) (ratio 12.5∶1).The age of disease onset was (59.5 ± 14.3) years.The most common presenting symptoms were jaundice and abdominal pain (both 59.3％).The most common complication was IgG4-related sclerosing cholangitis (63.0％).Magnetic Resonance Cholangiopancreatograhpy (MRCP) conducted on 26 patients showed the diffuse type 1 AIP was most common (53.8％),only 2 patients (7.7％) presented with a main pancreatic duct stricture.The pancreatic segment of bile duct narrowing was very common (84.6％),and most patients presented as tapered narrowings (65.4％).Standard glucocorticoid therapy was given to these 27 patients who responded well with clinical and laboratory remissions.Two patients were given maintenance glucocorticoid therapy for a high level of serum IgG4.Conclusions Type 1 AIP can present as a local manifestation of IgG4-related disease.The most common complication is IgG4-related sclerosing cholangitis.Glucocorticoid therapy was effective but some patients required maintenance therapy.

Simultaneous presentation of autoimmune pancreatitis and ulcerative colitis: a case report

Autoimmune pancreatitis type 2, an increasingly recognized etiology of pancreatitis in young patients without elevated IgG4. We report the case of a young woman admitted for acute pancreatitis, whose initial etiological record was negative. The evaluation of gravity by CT scan showed a slightly hipertrophic pancreas with the discovery of an aspect of ulcerative colitis "pancolitis". This association helped to guide the etiological diagnosis and treatment management. The patient was put on steroids with a rapidly favorable course, without recurrence and a 12-month follow-up.

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.

Immunoglobulin G4-related sclerosing cholangitis

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition of which IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC, namely, primary sclerosing cholangitis and cholangiocarcinoma, outline diagnostic criteria for IgG4-SC, provide insight into possible pathophysiological mechanisms underlying the disease and discuss short and long-term management options of this recently described disease.