Autoimmune Pancreatitis Presenting Research Articles

Uncommon Presentation of Autoimmune Pancreatitis With Refractory Ulcerative Colitis

Patients diagnosed with autoimmune pancreatitis (AIP) are at significantly higher risk for ulcerative colitis (UC), and the concurrent diagnoses continue to be a challenge to recognize. A previously healthy symptomatic 23-year-old man was diagnosed with type II AIP with UC and, despite aggressive treatment with steroids and infliximab, ultimately had a total colectomy. This patient case illustrates the association between AIP and UC and proposes that a concomitant diagnosis suggests a worse prognosis. Determining the relationship between these 2 pathologies and early diagnosis will allow physicians to escalate therapy more quickly and improve outcomes in patients.

Autoimmune pancreatitis presenting as obstructive jaundice – A diagnostic challenge

Autoimmune pancreatitis (AIP) is a rare form of pancreatitis which can mimic carcinoma head of the pancreas or distal cholangiocarcinoma, causing the diagnostic dilemma. A 64-year-old postmenopausal woman presented with jaundice, generalized itching, and intermittent low-grade fever for 2 weeks. On evaluation, she had a bulky pancreas on imaging and distal biliary stricture on magnetic resonance cholangiopancreatography. She was planned for Whipple's resection, but her serum immunoglobulin G4 (IgG4) was very high. She was started on oral high-dose steroids considering the possibility of AIP and had significant improvement after 2 weeks of treatment. AIP can mimic distal cholangiocarcinoma and can cause a diagnostic challenge in patients admitted with obstructive jaundice. The presence of high serum IgG4 level and treatment response to steroids help in making the diagnosis. This case reminds the readers to consider AIP in patients with obstructive jaundice who have imaging findings of distal cholangiocarcinoma to avoid unnecessary operations on patients without cancer.

S1658 Unusual Presentation of Autoimmune Pancreatitis With Adenocarcinoma of the Head of the Pancreas

S1658 Unusual Presentation of Autoimmune Pancreatitis With Adenocarcinoma of the Head of the Pancreas

S3484 Autoimmune Pancreatitis Presenting as a Newly Discovered Pancreatic Mass

S3484 Autoimmune Pancreatitis Presenting as a Newly Discovered Pancreatic Mass

A Case of Autoimmune Pancreatitis Presenting as a Deterioration in Glycemic Control in a Patient with Pre-Existing Type 2 Diabetes

The present article demonstrates an unusual case of type 1 autoimmune pancreatitis (AIP), focusing on the cause of deterioration in glycemic control and weight loss in a patient with pre-existing type 2 diabetes mellitus (DM). A 67-year-old man was diagnosed with type 2 DM 23 years prior and presented with weight loss of approximately 6 kg over a period of 3 months and hemoglobin A1c (HbA1c) of 9.1%. His carbohydrate antigen 19-9 (CA 19-9) level was elevated to 158.54 U/mL (normal, 0~37 U/mL). Considering these findings, we needed to rule out hidden malignancy. Computed tomography of the abdomen showed diffuse swelling of the pancreas uncinated process. The serum immunoglobulin G (IgG) level was elevated to 2,418 mg/dL (normal, 700~1,600 mg/dL), and IgG4 level was elevated to 1,115.0 mg/dL (normal range, 3.9~86.4 mg/dL). This case highlights that AIP should be considered as a cause of significant weight loss and a deterioration in glycemic control in patients with DM. Furthermore, a pancreatic imaging study should be considered in clinical practice to differentiate pancreatic cancer and AIP. Keywords: Autoimmune pancreatitis; Diabetes mellitus; type 2

The role of EUS in diagnosing focal autoimmune pancreatitis and differentiating it from pancreatic cancer.

Background and Objectives:The clinical presentation of focal autoimmune pancreatitis (FAIP) and together with radiological overlap can mimic pancreatic cancer (PC). The aim of this study is to construct and validate a prediction model for differentiating FAIP from PC according to EUS characteristics.Patients and Methods:Ninety patients with FAIP and 196 patients with PC, who consecutively underwent EUS at our center from January 2013 to December 2018, were retrospectively included in the study. The enrolled patients were randomly divided into either a derivation sample or a validation sample. According to EUS characteristics, multivariate stepwise logistic regression and receiver operating characteristics (ROC) analyses were used to construct a prediction model in derivation sample, and then, the efficacy was assessed in validation sample.Results:EUS characteristics that were suggestive of FAIP included diffuse hypoechogenicity, hyperechoic foci/stands or lobularity (parenchymal heterogeneity), bile duct wall thickening and peripancreatic hypoechoic margin; and EUS features favoring PC included focal hypoechogenicity, absence of parenchymal heterogeneity, pancreatic duct dilation, and vessel involvement. The prediction model, with an area under the ROC curve of more than 0.95, had a good capability to distinguish FAIP from PC. By using the optimal cutoff value, the efficacy of model for diagnosing PC showed 83.7%–91.8% sensitivity and 93.3%–95.6% specificity.Conclusions:It is feasible to differentiate FAIP from PC based on EUS characteristics. The prediction model built in this study needs to be further confirmed by multicenter prospective researches.

S3484 A Case of Ulcerative Proctitis in a Patient With Recent Diagnosis of Autoimmune Pancreatitis

INTRODUCTION: The association between autoimmune pancreatitis (AIP) and inflammatory bowel disease (IBD) is extremely rare and poorly understood. Autoimmune pancreatitis presenting in patients with IBD poses a diagnostic dilemma, as it is difficult to differentiate between AIP and primary sclerosing cholangitis. The relationship between IBD and AIP is mostly limited to UC. The following is a rare case of a patient with AIP and UC. CASE DESCRIPTION/METHODS: A 28-year-old man presented to our office for evaluation of abnormal liver function tests. Symptoms included jaundice and an episode of generalized abdominal pain two weeks before, which resolved spontaneously. Remarkable laboratory results included AST 158, ALT 355, ALP 281 and total bilirubin 4.76. MRCP revealed CBD dilation. Subsequent ERCP with cholangioscopy revealed a distal CBD stricture. A directed biopsy was obtained with deployment of a plastic biliary stent for relief of obstruction. Due to his young age and presentation with biliary stricture and a history of abdominal pain, AIP was suspected. IgG4 serology was found to be positive confirming the diagnosis. He was started on steroid therapy and presented later with rectal bleeding prompting colonoscopy evaluation revealing distal inflammation in the rectum with biopsies consistent with ulcerative proctitis. He was treated with oral and rectal mesalamine and a tapering dose of steroids. Two months later his symptoms resolved, and repeat ERCP revealed resolution of the CBD stricture. Follow-up liver enzymes normalized. DISCUSSION: While an association between AIP and concurrent immune/inflammatory diseases, especially UC has been reported, an exact relationship has not been ascertained. Type 2 AIP was recently identified to be associated with IBD. However, Type 2 usually presents in young patients with IgG4 negative serology. This case is unique as our patient is young but presented with IgG4 positive AIP.

S1579 Diagnosis of Autoimmune Pancreatitis via Fine Needle Biopsy and HISORt Criteria in a 43-Year-Old African American Male With an Atypical Presentation

INTRODUCTION: Autoimmune pancreatitis (AIP) is a rare disorder characterized by abnormal imaging, elevated IgG4 levels in a majority of cases, typical histology, and usually a perceptible response to steroids. It is further divided into two different subsets, Type 1 and Type 2, with different characteristics and histology. It is difficult to diagnose this condition via a fine needle aspiration (FNA) sample due to scant tissue retrieval; hence other diagnostic criteria like HISORt, as proposed by the Mayo Clinic, are often used. It utilizes histology, imaging, serology, other organ involvement, and response to steroid therapy. CASE DESCRIPTION/METHODS: We report an atypical presentation of autoimmune pancreatitis in a 43-year-old African American male who presented with abdominal pain, diarrhea, a 40 lb weight loss, pancreatic mass, and no known history of autoimmune diseases. Comorbidities include hypertension and diabetes mellitus. An EUS with Fine Needle Biopsy (FNB) was performed, which demonstrated an adequate sample with classic lymphoplasmacytic reaction, which stained positively for IgG4. Serum IgG4 levels were also elevated. A diagnosis of Type 1 AIP was made. The patient’s fecal elastase was low, denoting Exocrine Pancreatic Insufficiency (EPI). He was started on a prednisone taper, along with azathioprine 50 mg and weight-based Creon therapy. Six months post intervention, the patient is symptom free and has regained 15 lbs. DISCUSSION: This case demonstrates the value of the FNB sampling technique to obtain accurate and adequate tissue histology that is not only diagnostic but can further differentiate between Type 1 and 2 AIP, along with validation of the HISORt criteria. In addition, it demonstrates the value of screening for EPI in patients with AIP.Figure 1.: Diffusely thickened appearance of the pancreas and an ill-defined, bi-lobed cystic area at the tail, measuring 1 × 2cm, contiguous with the lower pole of the spleen. Mild splenomegaly and interval development of a slightly irregular cystic splenic mass, measuring 4.5 × 4.3cm.Figure 2.: A 4.8 × 4.1cm oval and hypoechoic lesion at the junction of the body and tail of the pancreas. Calcified mass with a cystic and necrotic component, concerning for a pseudocyst.Figure 3.: A: IgG4 staining, B: MUM-1 staining, C: Storiform fibrosis with lymphoplasmacytic infiltrate, D: Atrophic pancreatic acinar epithelium with fibrosis and inflammation.

Development of Pancreatic Adenocarcinoma in Autoimmune Pancreatitis: A Systematic Review

Introduction: Presentation of autoimmune pancreatitis (AIP) can be similar to pancreatic adenocarcinoma (PAC) with a pancreatic mass that may cause obstructive jaundice. Chronic pancreatitis predisposes patients to PAC. The relationship between AIP and PAC is unknown. The aim of this systematic review is to determine the frequency of incident PAC in AIP, and secondarily to assess for prevalent PAC and AIP. Methods: A systematic search using PubMed, Embase, Scopus & Cochrane was performed. Search criteria included “autoimmune pancreatitis,” “pancreatic cancer,” “adenocarcinoma,” & “carcinoma” with all permutations. AIP was defined by international guidelines. Our inclusion criterion for incident PAC cases was a minimum of 1 year between AIP and PAC diagnoses to exclude prevalent cancers. The inclusion criterion for prevalent PAC cases was defined by presence of AIP and PAC within 1 year. The search yielded 636 results reviewed by 2 authors. 8 case reports and 4 retrospective studies with a total of 19 patients were included in the combined analyses. Results: There were 7 cases of incident PAC, with 4/7 (57.1%) men, mean age 67±14.1yrs (Range 39-80yrs) (Table 1). Mean time from AIP to PAC diagnosis was 78.1±60.9months (Range 12-186 months). 6/7 (85.7%) patients received prednisone therapy for AIP. 12 total cases of prevalent PAC with AIP were identified, all of which were men, mean age 61.4±5yrs (Range 53-70yrs) (Table 2). All prevalent cases of PAC with AIP were diagnosed simultaneously, with pathologic confirmation of both entities present on histology in 11/12 cases. Medical treatment of AIP was not reported for any prevalent PAC cases. Stage of PAC at diagnosis when reported was similar between incident & prevalent cases with most cases being unresectable (60% vs. 66.7%). Conclusion: Development of PAC in underlying AIP is exceedingly rare with only 7 total incident cases reported in the literature. The absolute risk remains unknown and was unable to be calculated based on included study types in this analysis. The number of prevalent cases of PAC concomitantly diagnosed with AIP should prompt a comprehensive investigation to exclude the presence of underlying malignancy and should be performed in cases of newly diagnosed AIP. Further prospective studies are warranted to determine overall risk of PAC in AIP.2733_A Figure 1. Study Characteristics of Autoimmune Pancreatitis Patients who Developed Incident Pancreatic Adenocarcinoma2733_B Figure 2. Study Characteristics of Autoimmune Pancreatitis Patients who had Prevalent Pancreatic Adenocarcinoma

Autoimmune Pancreatitis in Two Adolescent Boys

In this current review, we report the cases of autoimmune pancreatitis in two adolescent boys. The diagnosis was made essentially on the basis of imaging result and response to steroid therapy. One of them developed ulcerative colitis 1 year later, which was in favor of the autoimmune pancreatitis diagnosis. Since the presentation of autoimmune pancreatitis can mimic other conditions, a prompt diagnosis should be made, helping the patient receive adequate treatment and avoid unnecessary surgical interventions. Int J Clin Pediatr. 2018;7(3):39-42 doi: https://doi.org/10.14740/ijcp303w

Autoimmune pancreatitis presenting as obstructive jaundice

A 71 years cachexic male presented with sign symptoms of biliary tract obstruction. There was a large mass in the head of pancreases with raised CA 19.9. But elevated serum lipase raised the suspicion of paocreatitis. Elevated immunoglobu­lin IgG4 confmned this case as a auto immune aetiology. Initially there was much difficulty to differentiate auto immune pancreatitis from pancreatic carcinoma but after successful stenting and a course of corticosteroids, patient improved dramatically along with nonnalisation of all the radiological, bio-chemical and immunological parameters.

Atypical presentation of IgG4-related autoimmune pancreatitis - be awake to early detecting subsequent renal involvement: case report and literature review

Atypical presentation of IgG4-related autoimmune pancreatitis - be awake to early detecting subsequent renal involvement: case report and literature review

Epidemiology, clinical presentation, diagnosis and treatment of autoimmune pancreatitis: A retrospective analysis of 53 patients.

Most of the data about epidemiology, clinical presentation and treatment of autoimmune pancreatitis (AIP) is based on case series or small study groups. We therefore analyzed all cases of AIP treated at our clinic retrospectively. We searched our clinical database for the diagnosis pancreatitis between January 2007 and June 2014, selected patients with AIP and entered all relevant information in a database for statistical analysis. In total 53 patients with AIP were treated at our institution, 62% with type 1 and 23% with type 2 AIP. Gender distribution was male/female 3.1:1 for type 1 and 1:1.2 for type 2 AIP. The median age was 63.0 and 32.5 years for type 1 and type 2 AIP, respectively. The most common symptom is abdominal pain particular in patients with type 2 AIP whereas jaundice was only apparent in patients with type 1 AIP. The international diagnostic criteria seem to facilitate diagnosis of AIP as unnecessary pancreatic surgery in patients with AIP decreases. In 62.6% of the patients therapy was indicated and 84.8% showed a response to initial therapy with steroids. Recurring disease occurred in 28.3% of the cases but only 3.8% suffered a second relapse. Permanent maintenance therapy with steroids or additional therapy with immunomodulatory drugs is successful in recurring disease. Our data further corroborate previous findings on epidemiology, clinical presentation and treatment of AIP. AIP is a well manageable autoimmune disease in most patients. Better biopsy techniques and simplified diagnostic criteria might further alleviate diagnosis of AIP.

CT and MRI Findings of Autoimmune Polymorph Bifocal Pancreatitis Mimicking Pancreatic Adenocarcinoma: A Case Report and Review of the Literature.

Autoimmune pancreatitis is a rare type of chronic pancreatitis. It is supposed to be a pancreatic manifestation of an immune-complex modulated systemic disorder. In contrast, pancreatic adenocarcinoma is the most frequent malignant neoplasm of the pancreas. Within the rare type of focal autoimmune pancreatitis, only few presentations with multifocal pancreatic lesions have been described. Herein we report a case of a 58-year-old patient with autoimmune pancreatitis presenting with bifocal manifestations of the pancreatic head and tail, mimicking pancreatic adenocarcinoma clinically, on computed tomography and magnetic resonance imaging. Typical imaging findings of autoimmune pancreatitis are compared with typical findings in pancreatic carcinoma. The diagnostic dilemma of differentiating between both entities is discussed. A review of the present literature regarding multifocal presence of autoimmune pancreatitis is performed.

Immunosuppressive drugs for the treatment of autoimmune pancreatitis.

Autoimmune pancreatitis is one of the few diseases of the pancreas characterized by the possibility of curing the illness using immunosuppressant drugs. In this paper, the therapeutic approach used to treat autoimmune pancreatitis patients and the clinical outcome related to each treatment modality were reviewed. Steroids are useful in alleviating the symptoms of the acute presentation of autoimmune pancreatitis, but some questions remain open, such as a shared definition of the disease's remission as well as autoimmune pancreatitis relapse, the dosage of steroids in the symptomatic phase of the disease and the duration of steroid therapy. Finally, it should be determined if other immunosuppressive nonsteroidal drugs could become first-line therapy in patients with autoimmune pancreatitis without jaundice and without atrophic pancreas.

Long‐term Surgical Outcomes of Patients with Type 1 Autoimmune Pancreatitis

Although pancreatectomy has sometimes been performed for patients with autoimmune pancreatitis (AIP) presenting atypical radiologic findings under the diagnosis of pancreatobiliary malignancy, the long-term surgical outcome of these patients had not yet been fully elucidated. The long-term surgical outcomes of 13 patients with pathologically diagnosed type 1 AIP with immunohistochemical staining for immunoglobulin G4 (IgG4) were retrospectively compared with those of 34 patients with conventional chronic pancreatitis to evaluate the residual pancreatic function. A definite relapse of AIP in terms of the clinical manifestations and diagnostic imaging was not found in any of the patients, although one patient experienced an attack of acute pancreatitis caused by pancreatic stones, and stricture of the hepaticojejunostomy occurred in one patient. The overall body weight decreased significantly more in patients with AIP than in patients with conventional chronic pancreatitis (p < 0.05); however, there was no difference in the preoperative nondiabetic patients. Refractory diarrhea occurred in only one patient with AIP. The cumulative new-onset rates of diabetes mellitus of the ten patients with AIP and 24 patients with conventional chronic pancreatitis at 5 years after the surgery were 32.5 and 26.1 %, respectively (p = 0.70). Careful long-term follow-up is needed for patients undergoing pancreatectomy for type 1 AIP because remnant pancreatic function can deteriorate as severely as that of patients who undergo pancreatectomy for conventional chronic pancreatitis. In the present series, however, there were few definite manifestations indicating relapse or the persistent existence of AIP.

Combination of CFTR Gene Mutation and Autoimmune Pancreatitis Presenting as Necrotizing Pancreatitis

Division of Pediatric Gastroenterology and Nutrition, Cohen Children’s Medical Center North Shore-Long Island Jewish Health System 269-01 76th Ave New Hyde Park, NY 11040 [email protected] Division of Pediatric Gastroenterology and Nutrition, Cohen Children’s Medical Center North Shore-Long Island Jewish Health System New Hyde Park, NY

Autoimmune Pancreatitis Presenting as Obstruction Jaundice

Autoimmune pancreatitis is a type of chronic pancreatitis characterized by an autoimmune inflammatory process in which prominent lymphocyte infiltration with associated fibrosis of the pancreas causes organ dysfunction. Clinically it is very important to be aware of autoimmune pancreatitis because autoimmune chronic pancreatitis (AIP) can clinically disguise as pancreaticobiliary malignancies, ordinary chronic or acute pancreatitis. Here we report a case of autoimmune pancreatitis presented with obstructive jaundice with initial diagnosis of carcinoma of head of the pancreas. DOI: http://dx.doi.org/10.3329/jbcps.v29i4.11344 J Bangladesh Coll Phys Surg 2011; 29: 231-234

Autoimmune pancreatitis mimicking carcinoma of the head of the pancreas: a case report

IntroductionWe report on a case of autoimmune pancreatitis presenting as pancreatic head cancer, which is extremely rare in Iran. Currently, on the PubMed database, no such cases exist.Case presentationA 70-year-old Iranian man presented with recurrent abdominal pain, jaundice and elevated bilirubin and alkaline phosphatase levels. An abdominal computed tomography scan revealed a heterogeneous presence in the pancreatic head as well as dilated intra- and extrahepatic bile ducts. A common bile duct stent had been inserted. Our patient was subsequently diagnosed with pancreatic head cancer.Due to his continued recurrent abdominal pain, our patient returned to the hospital. His levels of bilirubin, alkaline phosphatase and tumor markers were all normal but his immunoglobulin G4 and antinuclear antibodies were extremely high. A biopsy of the pancreatic head heterogeneity by endoscopic ultrasonography was performed.Pathologic samples showed fibrosis associated with lymphoplasmacytic infiltration and no evidence of malignancy. A diagnosis of autoimmune pancreatitis was confirmed, the bile duct stent removed, and an appropriate treatment plan was undertaken.ConclusionAutoimmune pancreatitis should be considered in suspected cases of pancreatic cancer. In these instances, a biopsy of the pancreas will help to differentiate between the two and prevent complications due to disease progression as well as unnecessary surgery.

Autoimmune Pancreatitis Presenting as a Focal Pancreatic Mass Mimicking Pancreatic Cancer: A Case Report and Review of the Literature

A 68-year-old female presented with a 2-week history of jaundice, generalized itching and weight loss in 2006. On physical examination, she was deeply jaundiced; however, other clinical examinations were completely unremarkable. Liver function tests (LFTs) were deranged: serum alkaline phosphatase 407 U/L (35 - 104), serum aspartate transaminase 57 U/L (0 - 31), serum alanine transaminase 184 U/L (0 - 37), and serum total bilirubin 4.3 mg/dL (0 - 1). Abdominal CT revealed a 4.8 cm × 3.5 cm mass in the pancreatic head. ERCP revealed a high-grade stricture in the distal CBD. An internal biliary stent was placed in to the CBD stricture. Patient’s symptoms relieved after biliary stenting. Serum CA 19-9 was elevated at 1,568 U/mL (0 - 37). Due to high suspicion for pancreatic cancer, patient underwent pylorus-sparing pancreaticoduodenectomy. Histopathology was benign and showed perilobular lymphoplasmacytic infiltrate, periductal fibrosis and phlebitis that was consistent with autoimmune pancreatitis. Patient was started on oral prednisone that was eventually replaced with azathioprine. Serum IgG4 was 53 mg/dL (1 - 291) after starting steroid treatment. J Med Cases. 2013;4(1):29-33 doi: https://doi.org/10.4021/jmc891w