Eosinophilic pancreatitis with serum IgG4-positivity, potentially associated with autoimmune pancreatitis.

A 70-year-old man presented with unprovoked weight loss and intermittent left upper quadrant tenderness for one-and-a-half month. Computed tomography revealed irregular cystic lesions in the pancreatic tail. Endoscopic ultrasonography (EUS) revealed pancreatic tail enlargement and giant, thick-walled cysts surrounding the pancreatic tail with no connection with the pancreatic duct. Endoscopic ultrasonography-guided fine-needle aspiration was performed on the enlarged pancreatic parenchyma and thickened cyst wall. Both biopsies showed hyper eosinophilia and few plasma cells. Endoscopic retrograde pancreatography revealed an irregular narrowing of the main pancreatic duct. Pancreatic juice cytology revealed substantial eosinophilia. Blood sampling showed an elevated eosinophil count and immunoglobulin G4 (IgG4) and immunoglobulin E (IgE) levels at the initial examination. We considered the patient to have eosinophilic pancreatitis (EP) with autoimmune pancreatitis, or alternately, EP with IgG4-related retroperitoneal fibrosis (RPF). Upon prednisolone administration, the abdominal pain improved, the peripheral blood eosinophil count decreased to zero, IgG4 and IgE levels decreased, pancreatic enlargement improved, and the cystic lesions disappeared. The condition did not recur within the following 3 years. Both EP and EP with IgG4-related RPF are rare etiologies of pancreatitis, and this case is very instructive.

Eosinophilic pancreatitis presenting as rupture of a pancreatic cystic lesion into the chest cavity.

A 71-year-old man was receiving follow-up examination because of a retention cyst in the pancreatic body that extended to the dorsal extrahepatic area, but presented to the Emergency Department at our hospital with dyspnea and cough. Chest X-ray showed a large amount of left-sided pleural effusion and abdominal computed tomography (CT) showed reduction in size of the cystic lesion. Biochemical testing of the pleural effusion revealed high levels of pancreatic enzymes. We, therefore, diagnosed rupture of the pancreatic cystic lesion into the chest cavity. Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated stenosis of the pancreatic duct and leakage of contrast medium at the cystic lesion. CT after ERCP revealed leakage of contrast medium from the cystic lesion through the dorsal extrahepatic area into the chest cavity. Endoscopic naso-pancreatic drainage was performed, but the cystic lesion and pleural effusion remained unimproved. Distal pancreatectomy was, therefore, performed. Microscopic examination revealed eosinophilic infiltration of the pancreatic parenchyma, leading to a diagnosis of eosinophilic pancreatitis (EP). Pancreatic retention cyst secondary to chronic pancreatitis associated with eosinophilic infiltration was considered to have ruptured into the chest cavity. EP is a rare etiology of pancreatitis and few cases have been reported. This case was thus considered valuable.

Eosinophilic pancreatitis presenting as possible malignancy.

Eosinophilic pancreatitis is a rare form of chronic pancreatitis that is often mistaken for malignancy due to nonspecific presenting symptoms and noncontributory auxiliary exams. This clinical case report highlights a unique presentation of chronic pancreatitis. A middle-aged man developed abdominal pain, and repeat imaging showed an interval increase in size of a known pancreatic lesion. A core biopsy showed benign pancreatic tissue with eosinophilic-rich inflammation; IgE levels were also elevated. The patient was started on a prednisone taper, and repeat computed tomography 3 months later showed a decreased size of the pancreatic lesion. Correct and timely diagnosis of eosinophilic pancreatitis may avoid invasive unnecessary surgical procedures and allow for earlier initiation of appropriate therapy.

Eosinophilic pancreatitis: a review of the pathophysiology, diagnosis, and treatment.

Eosinophilic pancreatitis (EP) is an extremely rare disease caused by purely eosinophilic infiltration of the pancreas. EP is prone to being misdiagnosed as pancreatic cancer, causing unnecessary economic and physical harm to the patient. We report three cases of EP that were cured by steroids without relapse from 2017 to now. The clinical data of the three patients, including clinical manifestations, serological manifestations, imaging (ultrasound, computed tomography, and MRI), pathological diagnosis and treatment, and telephone follow-up of all patients, were retrospectively analysed. In addition, a literature search was conducted on the Web of Science and PubMed databases using key terms related to EP, considering case reports with no restrictions on the date of publication or language. In conclusion, we analysed 19 cases and determined the diagnostic criteria for EP. The diagnostic algorithm for EP can be used to diagnose EP easily. We hope that our standards and algorithm can reduce the rate of misdiagnosis and contribute to clinical diagnosis and treatment. In addition, we expect to evaluate more EP cases to test our diagnostic criteria and design a systematic diagnostic flow chart.

Eosinophils in the pathogenesis of pancreatic disorders.

Eosinophils comprise approximately 1-4% of total blood leukocytes that reside in the intestine, bone marrow, mammary gland, and adipose tissues to maintain innate immunity in healthy individuals. Eosinophils have four toxic granules known as major basic protein (MBP), eosinophil cationic protein (ECP), eosinophil peroxidase (EPO), and eosinophil-derived neurotoxin (EDN), and upon degranulation, these granules promote pathogenesis of inflammatory diseases like allergy, asthma, dermatitis, and gastrointestinal disorders. Additionally, the role of eosinophils is underscored in exocrine disorders including pancreatitis. Chronic pancreatitis (CP) is an inflammatory disorder that occurs due to the alcohol consumption, blockage of the pancreatic duct, and trypsinogen mutation. Eosinophil levels are detected in higher numbers in both CP and pancreatic cancer patients compared with healthy individuals. The mechanistic understanding of chronic inflammation-induced pancreatic malignancy has not yet been reached and requires further exploration. This review provides a comprehensive summary of the epidemiology, pathophysiology, evaluation, and management of eosinophil-associated pancreatic disorders and further summarizes current evidence regarding risk factors, pathophysiology, clinical features, diagnostic evaluation, treatment, and prognosis of eosinophilic pancreatitis (EP) and pancreatic cancer.

Experimental Modeling of Eosinophil-Associated Diseases.

Eosinophils are an important subtype of leukocytes derived from bone marrow multipotent hematopoietic stem cells and represent about 1% of leukocytes in circulating blood. In homeostatic conditions, eosinophils reside in the intestine to maintain the balance of immune responses by communicating with gut microbes without causing inflammation. However, under the stressed or diseased condition, eosinophils degranulate, releasing their granule-derived cytotoxic proteins that are involved in inflammatory responses. Various eosinophil-associated inflammatory diseases are eosinophilic esophagitis (EoE), eosinophilic gastroenteritis (EG), and eosinophilic colitis (EC), together called EGID, asthma, hypereosinophilic syndrome, and eosinophilic pneumonia (EP). Eosinophil degranulation results in the release of their four toxic proteins [major basic protein (MBP), eosinophil cationic protein (ECP), eosinophil peroxidase (EPO), and eosinophil-derived neurotoxin (EDN)] which promote disease pathogenesis. Pancreatitis is the inflammatory disease of the pancreas that arises due to blockage of the pancreatic duct, trypsinogen mutation, alcohol consumption, and repeated occurrence of pancreatitis leading to chronic pancreatitis (CP); subsequently some CP patients may also develop pancreatic cancer. The presence of eosinophils is now shown in various case reports with acute, recurrent acute, and chronic pancreatitis and pancreatic cancer indicating the role of eosinophils in the pathogenesis of various pancreatic inflammatory disorders. However, the details of eosinophil accumulation during pancreatic diseases are not well explored and need further attention. Overall, the chapter provides the current understanding of reported eosinophils associated with inflammatory diseases like EGID diseases, asthma, and pancreatic disorders, i.e., acute, chronic pancreatitis, and pancreatic cancer. This knowledge will be helpful for future studies to develop novel treatment options for the eosinophils associated diseases. Therefore, more efforts are needed to perform preclinical and clinical studies in this field for the successful development of eosinophil-targeting treatments for a variety of eosinophil-associated diseases.

Hypereosinophilic Syndrome, Multiorgan Involvement and Response to Imatinib.

Hypereosinophilic syndrome (HES) is an uncommon syndrome characterized by peripheral blood eosinophils count of more than 1,500/mm3 with associated tissue damage. It can be either primary or secondary, for example, due to parasitic infections or inflammation. We present a case of a 49-year-old Asian female with recurrent hospital admissions for cholecystitis, gastritis, urinary cystitis, and pancreatitis. Her peripheral blood count showed excessive eosinophils 15,600-19,000/mm3 on different occasions. Pathology of her gallbladder and her gastric biopsies showed eosinophilic infiltration. Her bone marrow biopsy showed a normocellular marrow with active trilineage hematopoiesis, eosinophilia, mild megakaryocytic hyperplasia with a few atypical forms, and mild T-cell lymphocytosis. Flow cytometry showed no evidence of acute leukemia, or T-cell or B-cell lymphoproliferative disorder. On fluorescent in-situ hybridization (FISH), myeloproliferative neoplasms (MPN) testing was negative for platelet-derived growth factor receptor-alpha (PDGFRA), platelet-derived growth factor receptor-beta (PDGFRB), and fibroblast growth factor receptor-1 (FGFR1) rearrangement. Despite not having the FIP1L1-PDGFRA (factor interacting with PAPOLA and CPSF1-platelet-derived growth factor receptor, alpha polypeptide) gene fusion, our patient responded to the treatment with a significant decrease in her absolute eosinophils count and resolution of her symptoms.

Eosinophilic pancreatitis: a rare or unexplored disease entity?

Several case reports show accumulation of eosinophils in pancreatitis patients and term the disease as "eosinophilic pancreatitis (EP)". EP usually presents with a pancreatic tumour and abdominal pain in obstructive jaundice, which is generally not diagnosed until the patient undergoes pancreatic resection. Histologically, EP reveals distinct patterns like diffused, periductal, acinar, and septal inflammatory infiltrates with eosinophils, eosinophilic phlebitis, and localised extreme eosinophilic infiltrates related with pseudocyst formation. EP patients also have elevated serum IgE levels with high eosinophil counts in the pancreas as well as in other organs such as the gastrointestinal tract, which is termed as eosinophilic gastroenteritis. Due to the lack of knowledge based on just a few case reports, it is considered that eosinophilic infiltration is quite rare in the pancreas; therefore, the significance of eosinophils in pancreatitis is not yet established. This review assesses the current understanding of eosinophilic pancreatitis and the important role of eosinophils in promoting pancreatic fibrosis including malignancy.

Progress in eosinophilic pancreatitis of children / 国际儿科学杂志

Eosinophilic pancreatitis ( EP) is one type of the very rare diseases in children with non-specific clinical pancreatic allergic features. The diagnosis cannot be made unless the histology of the pancreatic tissue. This review summarizes the characteristics and mechanism, imaging findings, diagnostric criteria and differential diagnosis, as well as the treatment of EP in children.

Eosinophilic Pancreatitis: A Rare Cause of Recurrent Acute Pancreatitis.

Eosinophilic pancreatitis is a rare form of recurrent acute pancreatitis that demonstrates distinct histologic features, including diffuse, periductal, acinar, and septal inflammatory infiltrates comprised of a pure or predominant population of eosinophils, eosinophilic phlebitis and arteritis, and localized eosinophilic infiltrates with pseudocyst formation. It is associated with elevated serum immunoglobulin E levels, an elevated eosinophil count with systemic manifestations, and eosinophilic infiltrates in other organs of the gastrointestinal tract. We present a case of eosinophilic pancreatitis in a 44-year-old man who was diagnosed after pancreatic resection for recurrent bouts of acute pancreatitis. While the gross and histologic evaluations matched other reported cases of eosinophilic pancreatitis, our patient had only minimal peripheral eosinophilia, no reported history of symptoms related to elevated eosinophilia or immunoglobulin E, and only mild eosinophilic infiltrates in his gallbladder.

Eosinophilic pancreatitis: Three case reports and literature review.

Eosinophilic pancreatitis (EP) is a rare form of chronic pancreatitis characterized by localized or diffuse eosinophilic infiltration of the pancreas and elevated serum immunoglobulin E levels. EP is difficult to distinguish from pancreatic cancer on the basis of clinical symptoms and the results of auxiliary examination alone. A retrospective analysis of the clinicopathological characteristics and laboratory, imaging, and pathology results of 3 patients with EP, who were initially diagnosed with pancreatic malignancy, was performed. EP is an allergic disease with non-specific clinical manifestations that is difficult to distinguish from pancreatic cancer based exclusively on clinical symptoms and auxiliary examination, resulting in the need for invasive procedures to confirm the diagnosis. An increase in the eosinophil count in the peripheral blood and pathological examination are essential for the diagnosis of EP.

A Unique Case of Eosinophilic Pancreatitis and Anencephaly in the Fetus of a Type I Diabetic Mother.

Pancreatic infiltration with eosinophils is an uncommon finding with numerous etiologies. While two rare cases of eosinophilic pancreatitis in infants born to Type I diabetic mothers have been reported once in the English literature and once in the French literature, we present the additional finding of anencephaly in a 34 week old fetus. Although the pancreas was grossly unremarkable, histological inspection demonstrated an eosinophilic infiltrate in the fibrous septae and islets of Langerhans along with hypertrophy and hyperplasia of the pancreatic islets.

A Case of Eosinophilic Pancreatitis / 대한소화기학회지

Eosinophilic pancreatitis is a rare disorder that may only be diagnosed after pancreatic resection under the suspection of a pancreatic tumor. We experienced a 65-year-old female patient whose initial presentation suggested pancreatic cancer. Radiologic evaluation revealed a pancreatic mass-like lesion which was obstructing the main pancreatic duct. Endoscopic retrograde cholangiopancreatography (ERCP) showed double duct strictures involving the distal common bile duct and the main pancreatic duct. Serum IgE level was elevated. Percutaneous core needle biopsy with an 18-gauge needle was performed targeting the pancreatic lesion. The biopsy specimen revealed fibrotic interlobular septum and intralobular fibrosis with prominent eosinophilic infiltration. The patient was treated with oral prednisolone (40 mg/day). A plastic stent was inserted into the narrowed common bile duct. After three months of oral steroid therapy, symptoms and signs improved rapidly and serum IgE level was decreased. Abdominal computed tomography and ERCP revealed remission of pancreatic mass-like lesion.