Pancreatic Solid Focal Lesions: Differential Diagnosis between Autoimmune Pancreatitis and Pancreatic Cancer

Background: Diagnosis of pancreatic cancer (PC) in early stages is still challenging for gastroenterologists. The early detection of cancer is one of the utmost importance for the successful therapy of this malignancy. An accurate differential diagnosis of focal pancreatic lesions plays also an important role, whether it is differential diagnosis of chronic pancreatitis from PC or autoimmune pancreatitis (AIP) from PC. Raised serum immunoglobulin G4 (IgG4) levels to twice the normal value are considered one of significant diagnostic features of type 1 AIP. However, IgG4 can be increased also in patients with PC, but levels usually do not exceed twice the normal value. Methods: In years 2012–2017, IgG4 serum levels were examined in 115 patients with histologically confirmed PC. Patients with PC and elevated IgG4 level (above 135 mg/dL) had tested their histological resection specimens or bioptic specimens from pancreatic lesion, with targeted detection of the presence of IgG4 and plasmocytes in the pancreatic tissue and changes characteristic for type 1 AIP. Results: A plasmatic IgG4 level in 115 patients with diagnosed PC was higher than 135 mg/dL in 14 patients (12.2%). Out of them, 2 patients (1.7%) revealed a serum IgG4 level higher than double the normal value, that is, higher than 270.0 mg/dL (suggestive of AIP). One patient met histological criteria for diagnosis of AIP in the simultaneous presence of PC. Conclusion: Diagnosis of early cancer stages, particularly differentiating AIP from PC can be sometimes problematic. IgG4 levels can be slightly elevated also in case of PC. A targeted biopsy of the pancreas is the method of choice in cases suspected from a focal form of AIP and we recommend to prefer it over other modalities, such as, for example, response to steroid therapy.

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Localized autoimmune pancreatitis mimicking pancreatic cancer: Case report and literature review

Journal of International Medical Research ◽

10.1177/0300060517742303 ◽

2018 ◽

Vol 46 (4) ◽

pp. 1657-1665 ◽

Cited By ~ 6

Author(s):

Wen-Ling Hsu ◽

Shu-Min Chang ◽

Pei-Yin Wu ◽

Chin-Chuan Chang

Keyword(s):

Pancreatic Cancer ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Pancreatic Head ◽

Corticosteroid Treatment ◽

Cancer Case ◽

Pancreatic Lesion ◽

Immunoglobulin G4 ◽

Positron Emission ◽

Serum Igg4

Autoimmune pancreatitis (AP) is a rare autoimmune pancreatic manifestation of systemic immunoglobulin G4 (IgG4)-related sclerosing disease. Distinguishing between AP and pancreatic cancer is crucial because the clinical courses, treatments, and prognoses of these two disease entities are quite different. We herein report a case involving a 52-year-old man with subacute epigastralgia who visited our hospital for evaluation of a suspicious pancreatic mass found during esophagogastroduodenoscopy. Enhanced computed tomography (CT) revealed an enlarged lesion in the pancreatic head with encasement of hepatic vessels. The lesion also exhibited increased 18F-fluorodeoxyglucose accumulation on positron emission tomography/CT imaging, which was highly suggestive of pancreatic cancer. After open biopsy, morphologic examination showed an inflammatory infiltrate in the pancreas, which was compatible with chronic sclerotic pancreatitis. Further laboratory tests revealed an elevated serum IgG4 level, and the diagnosis of sclerotic pancreatitis was then confirmed. After corticosteroid treatment, the pancreatic lesion showed shrinkage on follow-up CT, and the serum IgG4 titer decreased to the normal range. This case suggests that clinicians should be familiar with the clinical presentations and diagnostic criteria of AP versus pancreatic cancer. An awareness of the differences between these diseases may avoid misdiagnosis and unnecessary surgical intervention.

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Immunoglobulin G4, Autoimmune Pancreatitis and Pancreatic Cancer

Digestive Diseases ◽

10.1159/000368337 ◽

2014 ◽

Vol 33 (1) ◽

pp. 86-90 ◽

Cited By ~ 3

Author(s):

Martina Bojková ◽

Petr Dítě ◽

Jana Dvořáčková ◽

Ivo Novotný ◽

Katarina Floreánová ◽

...

Keyword(s):

Pancreatic Cancer ◽

Steroid Therapy ◽

Autoimmune Pancreatitis ◽

Normal Limit ◽

Serum Levels ◽

Pancreatic Tissue ◽

Immunoglobulin G4 ◽

Related Disease ◽

Igg4 Related Disease ◽

Igg4 Level

Background: Immunoglobulin G4 (IgG4)-related diseases are a group of diseases characterized by enlargement of the affected organs, elevation of serum IgG4, massive infiltration of affected organs with lymphocytes and plasma cells with IgG4 positivity and tissue fibrosis. Type I autoimmune pancreatitis is one form of IgG4-related disease. For IgG4-related diseases, various localizations are described for up to 10% of malignancies. The aim of our study was to examine IgG4 serum levels and pancreatic tissue with respect to the simultaneous presence of autoimmune pancreatitis in patients with pancreatic cancer. Methods: IgG4 serum levels were examined In 106 patients with histologically confirmed pancreatic cancer. The level of 135 mg/dl was considered as the normal value. Pancreatic tissue was histologically examined with respect to the presence of markers of autoimmune pancreatitis. Results: A higher IgG4 level than the cut-off value of 135 mg/dl was proven in 11 patients with pancreatic cancer. Of these 11 patients, 7 had levels twice the normal limit (65.6%). Autoimmune pancreatitis was diagnosed in these individuals. In the case of 1 patient, it was basically an unexpected finding; another patient was initially diagnosed with autoimmune pancreatitis. Repeated biopsy of the pancreas at the time of diagnosis did not confirm the presence of tumour structures, therefore steroid therapy was started. At a check-up 6 months after starting steroid therapy, the condition of the patient improved subjectively and IgG4 levels decreased. However, endosonographically, malignancy was suspected, which was subsequently confirmed histologically. This patient also demonstrated an IgG4 level twice the normal limit. Conclusion: IgG4-related diseases can be accompanied by the simultaneous occurrence of malignancies, which also applies to autoimmune pancreatitis. Chronic pancreatitis is considered a risk factor for pancreatic cancer. It cannot be reliably confirmed whether this also applies to autoimmune pancreatitis. In accordance with other works, however, it is evident that, despite the described high sensitivity and specificity for IgG4 elevation in the case of autoimmune pancreatitis, even levels twice the normal limit are demonstrable in some individuals with pancreatic cancer, without the presence of autoimmune pancreatitis. We believe that patients with IgG4-related disease, including autoimmune pancreatitis, must be systematically monitored with respect to the potential presence of malignancy.

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Hepatobiliary and Pancreatic: Pancreatic cancer with elevated serum IgG4 level due to multiple myeloma mimicking localized autoimmune pancreatitis

Journal of Gastroenterology and Hepatology ◽

10.1111/jgh.14088 ◽

2018 ◽

Vol 33 (7) ◽

pp. 1310-1310 ◽

Cited By ~ 3

Author(s):

S Kato ◽

M Kuwatani ◽

K Kawakubo ◽

R Sugiura ◽

K Hirata ◽

...

Keyword(s):

Pancreatic Cancer ◽

Multiple Myeloma ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

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Serum Immunoglobulin G Fraction 4 Levels in Pancreatic Cancer: Elevations Not Associated With Autoimmune Pancreatitis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2008-132-48-sigfli ◽

2008 ◽

Vol 132 (1) ◽

pp. 48-53 ◽

Cited By ~ 8

Author(s):

Amit Raina ◽

Alyssa M. Krasinskas ◽

Julia B. Greer ◽

Janette Lamb ◽

Erin Fink ◽

...

Keyword(s):

Pancreatic Cancer ◽

Cancer Patients ◽

Immunoglobulin G ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Immunoglobulin ◽

Pancreatic Mass ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

Abstract Context.—Autoimmune pancreatitis is an uncommon, inflammatory disease of the pancreas that presents with clinical features, such as painless jaundice and a pancreatic mass, similar to those caused by pancreatic cancer. Patients with autoimmune pancreatitis frequently have elevated serum immunoglobulin G fraction 4 (IgG4) levels, and their pancreatic tissue may show IgG4-positive plasma cell infiltration. It is imperative to differentiate autoimmune pancreatitis from pancreatic cancer because autoimmune pancreatitis typically responds to corticosteroid treatment. A previous Japanese study reported that serum IgG4 greater than 135 mg/dL was 97% specific and 95% sensitive in predicting autoimmune pancreatitis. Objective.—To prospectively measure serum IgG4 levels in pancreatic cancer patients to ascertain whether increased levels might be present in this North American population. Design.—We collected blood samples and phenotypic information on 71 consecutive pancreatic cancer patients and 103 healthy controls who visited our clinics between October 2004 and April 2006. IgG4 levels were determined using a single radial immunodiffusion assay. A serum IgG4 level greater than 135 mg/dL was considered elevated. Results.—Five cancer patients had IgG4 elevation, with a mean serum IgG4 level of 160.8 mg/dL. None of our cancer patients with plasma IgG4 elevation demonstrated evidence of autoimmune pancreatitis. One control subject demonstrated elevated serum IgG4 unrelated to identified etiology. Conclusions.—As many as 7% of patients with pancreatic cancer have serum IgG4 levels above 135 mg/dL. In patients with pancreatic mass lesions and suspicion of cancer, an IgG4 level measuring between 135 and 200 mg/dL should be interpreted cautiously and not accepted as diagnostic of autoimmune pancreatitis without further evaluation.

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Morphologic endoscopic ultrasound features in the differential diagnosis between type 1 focal autoimmune pancreatitis and pancreatic cancer

Pancreatology ◽

10.1016/j.pan.2021.05.237 ◽

2021 ◽

Vol 21 ◽

pp. S87-S88

Author(s):

M. Tacelli ◽

P. Zaccari ◽

G. Capurso ◽

M. Lanzillotta ◽

E. Della Torre ◽

...

Keyword(s):

Pancreatic Cancer ◽

Differential Diagnosis ◽

Endoscopic Ultrasound ◽

Autoimmune Pancreatitis ◽

Ultrasound Features

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Autoimmune Pancreatitis Type 1 Associated with a Pancreatic Pseudocyst

Case Reports in Gastroenterology ◽

10.1159/000499444 ◽

2019 ◽

Vol 13 (1) ◽

pp. 195-199

Author(s):

Felix Hesse ◽

Rickmer Braren ◽

Roland M. Schmid ◽

Veit Phillip

Keyword(s):

Differential Diagnosis ◽

Autoimmune Pancreatitis ◽

Pancreatic Pseudocyst ◽

Elevated Serum ◽

Response To Treatment ◽

Cystic Lesions ◽

Serum Igg4 ◽

Underlying Pathology ◽

Tomography Scan

Pancreatic cystic lesions comprise diverse entities with different histopathological characteristics. Differential diagnosis is often challenging. Autoimmune pancreatitis (AIP) is usually not considered an underlying pathology in the differential diagnosis of peri-/pancreatic pseudo-/cystic lesions. We report the case of a 73-year-old male with diffuse pancreatic enlargement and an adjacent cystic lesion (60 × 80 mm) on computed tomography scan. Based on these imaging findings and an elevated serum IgG4 concentration, AIP complicated by a pancreatic pseudocyst was diagnosed, and treatment with glucocorticoids was started. Regular follow-ups showed a good response to treatment with regression of the pancreatic pseudocyst and remittent pancreatic swelling.

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AF.149 SIGNATURE ENDOSCOPIC ULTRASOUND FEATURES ARE HELPFUL IN THE DIFFERENTIAL DIAGNOSIS BETWEEN TYPE 1 FOCAL AUTOIMMUNE PANCREATITIS AND PANCREATIC CANCER: A PROOF-OF-CONCEPT STUDY

Digestive and Liver Disease ◽

10.1016/s1590-8658(21)00712-x ◽

2021 ◽

Vol 53 ◽

pp. S204

Author(s):

M. Tacelli ◽

P. Zaccari ◽

M.C. Petrone ◽

E. Della Torre ◽

M. Lanzillotta ◽

...

Keyword(s):

Pancreatic Cancer ◽

Differential Diagnosis ◽

Endoscopic Ultrasound ◽

Autoimmune Pancreatitis ◽

Proof Of Concept ◽

Concept Study ◽

Ultrasound Features

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Untypical autoimmune pancreatitis and pancreatic cancer: differential diagnosis experiences extracted from misdiagnose of two cases

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-019-1217-z ◽

2019 ◽

Vol 14 (1) ◽

Author(s):

Gaopeng Li ◽

Ting Liu ◽

Jian Zheng ◽

Wenqin Kang ◽

Jun Xu ◽

...

Keyword(s):

Pancreatic Cancer ◽

Autoimmune Pancreatitis ◽

Diagnostic Value ◽

Warning Signs ◽

Clear Cut ◽

Clinical Presentations ◽

Pet Ct ◽

Serum Igg4 ◽

Work Up ◽

Rare Diagnosis

Abstract Background Differentiation between pancreatic cancer (PC) and focal form of autoimmune pancreatitis (AIP) is very challenging, with similar clinical presentations, laboratory results and morphologic imagings of US, CT, EUS, MRI, ERCP, PET-CT. Even serum IgG4 and biopsy sometimes cannot give clear-cut differential accurate diagnostis. Considering the totally different management strategy of the two diseases, accurate diagnostic value is urgently needed to remind the clinicians of the rare diagnosis of untypical AIP among frequent PC-suspected patients. Results We present 2 laparotomy cases of AIP that had a high similar characteristic to PC and retrospectively extracted the warning signs that may help select untypical AIP in PC-suspected patients. Conclusions We find that mild fluctuating jaundice with abdominal pain, young age, tumor marker of TPS, TPA and diverse results between variable radiological tests can help to differentiate AIP mass from PC, through retrospectively analyzing work-up process of AIP in two patients who underwent laparotomy for suspected PC.

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