Autoimmune Pancreatitis with Normal Serum IgG4 Concentrations: What Is the Correct Classification for Such Patients?

Background. Autoimmune pancreatitis (AIP) often mimics pancreatic cancer. The diagnosis of both conditions is difficult preoperatively let alone when they coexist. Several reports have been published describing pancreatic cancer in the setting of AIP.Case Report. The case of a 53-year-old man who presented with abdominal pain, jaundice, and radiological features of autoimmune pancreatitis, with a “sausage-shaped” pancreas and bulky pancreatic head with portal vein impingement, is presented. He had a normal serum IgG4 and only mildly elevated Ca-19.9. Initial endoscopic ultrasound-(EUS-) guided fine-needle aspiration (FNA) of the pancreas revealed an inflammatory sclerosing process only. A repeat EUS guided biopsy following biliary decompression demonstrated both malignancy and features of autoimmune pancreatitis. At laparotomy, a uniformly hard, bulky pancreas was found with no sonographically definable mass. A total pancreatectomy with portal vein resection and reconstruction was performed. Histology revealed adenosquamous carcinoma of the pancreatic head and autoimmune pancreatitis and squamous metaplasia in the remaining pancreas.Conclusion. This case highlights the diagnostic and management difficulties in a patient with pancreatic cancer in the setting of serum IgG4-negative, Type 2 AIP.

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Identification of serum IFN-α and IL-33 as novel biomarkers for type 1 autoimmune pancreatitis and IgG4-related disease

Scientific Reports ◽

10.1038/s41598-020-71848-4 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Kosuke Minaga ◽

Tomohiro Watanabe ◽

Akane Hara ◽

Ken Kamata ◽

Shunsuke Omoto ◽

...

Keyword(s):

Autoimmune Pancreatitis ◽

Characteristic Curve ◽

Elevated Serum ◽

Normal Serum ◽

Related Disease ◽

Igg4 Related Disease ◽

Number Of Patients ◽

Serum Igg4 ◽

Type 1 Autoimmune Pancreatitis

Abstract IgG4-related disease (IgG4-RD) is a multi-organ autoimmune disease characterized by elevated serum IgG4 concentration. Although serum IgG4 concentration is widely used as a biomarker for IgG4-RD and type 1 autoimmune pancreatitis (AIP), a pancreatic manifestation of IgG4-RD, a significant number of patients have normal serum IgG4 levels, even in the active phase of the disease. Recently, we reported that the development of experimental AIP and human type 1 AIP is associated with increased expression of IFN-α and IL-33 in the pancreas. In this study, we assessed the utility of serum IFN-α and IL-33 levels as biomarkers for type 1 AIP and IgG4-RD. Serum IFN-α and IL-33 concentrations in patients who met the diagnostic criteria for definite type 1 AIP and/or IgG4-RD were significantly higher than in those with chronic pancreatitis or in healthy controls. Strong correlations between serum IFN-α, IL-33, and IgG4 concentrations were observed. Diagnostic performance of serum IFN-α and IL-33 concentrations as markers of type 1 AIP and/or IgG4-RD was comparable to that of serum IgG4 concentration, as calculated by the receiver operating characteristic curve analysis. Induction of remission by prednisolone treatment markedly decreased the serum concentration of these cytokines. We conclude that serum IFN-α and IL-33 concentrations can be useful as biomarkers for type 1 AIP and IgG4-RD.

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Su1386 Consideration of the Serum IgG4 Levels and EUS-FNA in Diagnosing Autoimmune Pancreatitis: Focusing on the Normal Serum IgG4 Cases

Gastrointestinal Endoscopy ◽

10.1016/j.gie.2011.03.439 ◽

2011 ◽

Vol 73 (4) ◽

pp. AB250

Author(s):

Takuya Ishikawa ◽

Yoshiki Hirooka ◽

Akihiro Itoh ◽

Hiroki Kawashima ◽

Eizaburo Ohno ◽

...

Keyword(s):

Autoimmune Pancreatitis ◽

Normal Serum ◽

Serum Igg4

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The coexistence of autoimmune pancreatitis and elevated serum IGG4 in autoimmune diseases

Zeitschrift für Gastroenterologie ◽

10.1055/s-0031-1278521 ◽

2011 ◽

Vol 49 (05) ◽

Author(s):

V Terzin ◽

I Földesi ◽

L Kovács ◽

G Pokornyi ◽

T Wittmann ◽

...

Keyword(s):

Autoimmune Diseases ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Igg4

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EFFECT OF STEROID THERAPY ON THE SERUM IgG4 LEVELS AND THE CLINICAL MANIFESTATIONS OF AUTOIMMUNE PANCREATITIS

Pancreas ◽

10.1097/01.mpa.0000335395.75884.a3 ◽

2008 ◽

Vol 37 (4) ◽

pp. 497 ◽

Cited By ~ 1

Author(s):

D. Suzuki ◽

K. Shimizu ◽

K. Shiratori

Keyword(s):

Steroid Therapy ◽

Autoimmune Pancreatitis ◽

Clinical Manifestations ◽

Serum Igg4

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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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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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