scholarly journals Treatment of Autoimmune Pancreatitis with the Anecdotes of the First Report

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Terumi Kamisawa ◽  
Tadashi Takeuchi
Keyword(s):  
Maintenance Therapy ◽  
Steroid Therapy ◽  
Autoimmune Pancreatitis ◽  
Elevated Serum ◽  
Oral Prednisolone ◽  
Bile Duct Stenosis ◽  
Glucose Levels ◽  
First Case ◽  
Serum Igg4 ◽  
Duct Stenosis

The first case that led researchers to put forward a new concept of autoimmune pancreatitis (AIP) was treated with steroids by gastroenterologists in Tokyo Women’s Medical University. It is important to differentiate AIP from pancreatic cancer before treatment with steroids is started. Today, steroids are standard therapy for AIP worldwide. In the Japanese consensus guidelines, steroid therapy is indicated for symptomatic AIP. After management of glucose levels and obstructive jaundice, oral prednisolone is initiated at 0.6 mg/kg/day for 2–4 weeks and is gradually tapered to a maintenance dose of 2.5–5 mg/day over 2-3 months. To prevent relapse, maintenance therapy with low-dose prednisolone is used. For relapsed AIP, readministration or increased doses of steroids are effective. The presence of proximal bile duct stenosis and elevated serum IgG4 levels may be predictive of relapse of AIP. It is necessary to verify the validity of the Japanese regimen of steroid therapy for AIP. The necessity, drugs, and duration of maintenance therapy for AIP need to be clarified by prospective studies.

scholarly journals Usefulness of Steroid Administration for Diagnosis of IgG4-Related Sclerosing Cholangitis

2012 ◽  
Vol 97 (2) ◽  
pp. 145-149 ◽  
Author(s):  
Norio Kubo ◽  
Hideki Suzuki ◽  
Tsutomu Kobayashi ◽  
Kenichiro Araki ◽  
Shigeru Sasaki ◽  
...  
Keyword(s):  
Bile Duct ◽  
Steroid Therapy ◽  
Sclerosing Cholangitis ◽  
Liver Volume ◽  
Immunoglobulin G4 ◽  
Steroid Administration ◽  
Bile Duct Stenosis ◽  
Serum Igg4 ◽  
Hilar Bile Duct ◽  
Duct Stenosis

Abstract Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is one of the IgG4-related systemic sclerosing diseases and responds well to steroid therapy. A 58-year-old male was admitted with hilar bile duct stenosis revealed by computed tomography. We performed percutaneous transhepatic right portal vein embolization (PTPE) and scheduled a right hepatectomy because a hilar cholangiocarcinoma was first suspected. However, there was no cytologic evidence of malignancy and serum IgG4 was elevated. Steroid therapy was initiated after PTPE. There was no evidence of bile duct stenosis after 4 weeks. Improving diagnostic technique, IgG4-SC was diagnosed and treated with steroid therapy. In some cases, we couldn't deny the malignancy and performed unnecessary resection. We recommend that steroid administration while waiting for the liver volume to increase after PTPE is useful. The therapy aids in the diagnosis of bile duct stenosis, which has value for a hilar bile duct limit type of IgG4-SC, as in the case reported here.

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

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

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

2018 ◽  
Vol 46 (4) ◽  
pp. 1657-1665 ◽  
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.

scholarly journals Immunoglobulin G4-related hypertrophic pachymeningitis

2019 ◽  
Vol 6 (4) ◽  
pp. e568 ◽  
Author(s):  
Michaël Levraut ◽  
Mikaël Cohen ◽  
Saskia Bresch ◽  
Caroline Giordana ◽  
Fanny Burel-Vandenbos ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Case Reports ◽  
Treatment Algorithm ◽  
Elevated Serum ◽  
Case Series ◽  
Hypertrophic Pachymeningitis ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Meningeal Involvement ◽  
Serum Igg4

ObjectiveMeningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).MethodsTwo IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.ResultsForty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.Discussion/conclusionIgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.

scholarly journals Multifocal Mass Lesions in Autoimmune Pancreatitis

2017 ◽  
Vol 11 (3) ◽  
pp. 686-693 ◽  
Author(s):  
Kazuhiro Suzumura ◽  
Etsuro Hatano ◽  
Naoki Uyama ◽  
Toshihiro Okada ◽  
Yasukane Asano ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Autoimmune Pancreatitis ◽  
Malignant Tumors ◽  
Histopathological Examination ◽  
Elevated Serum ◽  
Pancreatic Head ◽  
Pancreatic Ducts ◽  
Diffuse Infiltration ◽  
Serum Igg4 ◽  
Mass Lesions

A 59-year-old male patient with jaundice was referred to our hospital because of mass lesions in the pancreatic head and tail. An immunological examination revealed an elevated serum IgG4 level. Computed tomography showed two clear boundary mass lesions in the pancreatic head and tail. Magnetic resonance imaging showed that the mass lesions exhibited low intensity on T1-weighted images and iso-intensity on T2-weighted images. Magnetic resonance cholangiopancreatography showed an obstruction of the main pancreatic duct in the pancreatic head and tail. The possibility of malignant tumors could not be ruled out; therefore, we performed total pancreatectomy. A histopathological examination of the nodular lesions revealed severe lymphoplasmacytic infiltration and inflammatory change around the pancreatic ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the nodules. According to these pathological findings, we diagnosed the patient with IgG4-related multifocal mass lesions of autoimmune pancreatitis (AIP). It is difficult to distinguish between focal type AIP and pancreatic cancer. We herein report a rare case of multifocal mass lesions in AIP and include bibliographical comments.

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

2008 ◽  
Vol 132 (1) ◽  
pp. 48-53 ◽  
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.

Recurrent Autoimmune Pancreatitis with Heterotopic Bile Duct Stenosis

Pancreas ◽  
2010 ◽  
Vol 39 (5) ◽  
pp. 699
Author(s):  
Yutaka Okamoto ◽  
Yusuke Tando ◽  
Hiroyasu Kawabe ◽  
Shinsaku Fukuda
Keyword(s):  
Bile Duct ◽  
Autoimmune Pancreatitis ◽  
Bile Duct Stenosis ◽  
Duct Stenosis

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

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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