scholarly journals A Rare Multifocal Pattern of Type 2 Autoimmune Pancreatitis with Negative IgG4: A Potential Diagnostic Pitfall That May Mimic Multifocal Pancreatic Adenocarcinoma

2018 ◽  
Vol 12 (1) ◽  
pp. 46-55 ◽  
Author(s):  
Partha Hota ◽  
Tejas Patel ◽  
Xiaofeng Zhao ◽  
Nirag Jhala ◽  
Omar Agosto
Keyword(s):  
Acute Pancreatitis ◽  
Mr Imaging ◽  
Pancreatic Adenocarcinoma ◽  
Autoimmune Pancreatitis ◽  
Diagnostic Pitfall ◽  
First Case ◽  
Imaging Characteristics ◽  
Sclerosing Pancreatitis

Autoimmune pancreatitis (AIP) is an increasingly recognized form of acute pancreatitis characterized by obstructive jaundice with a rapid and dramatic treatment response to steroid therapy. Recently, AIP has been divided into two distinct phenotypes: lymphoplasmocytic sclerosing pancreatitis AIP (type 1) and idiopathic duct-centric pancreatitis AIP (type 2); each of which have their own distinct demographics, diagnostic criteria, and histopathological features. We report, to the best of our knowledge, the first case of a multifocal pattern of type 2 AIP characterized with both CT and MR imaging. This rare imaging pattern of AIP may mimic the appearance of more worrisome malignant etiologies such as multifocal pancreatic adenocarcinoma or lymphoma, with overlapping imaging characteristics potentially complicating or delaying diagnosis. Therefore, recognition of this atypical pattern of AIP and avoidance of this potential diagnostic pitfall is crucial.

scholarly journals Autoimmune Pancreatitis Type 2: Case Report

2017 ◽  
Vol 5 (4) ◽  
pp. 232470961773424 ◽  
Author(s):  
Chidinma Onweni ◽  
Harika Balagoni ◽  
Jennifer M. Treece ◽  
Emmanuel Addo Yobo ◽  
Archi Patel ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Acute Pancreatitis ◽  
Case Report ◽  
Autoimmune Pancreatitis ◽  
Corticosteroid Treatment ◽  
Unknown Etiology ◽  
Inflammatory Bowel ◽  
Igg4 Antibody

A middle-aged man presents with acute pancreatitis of unknown etiology and is found to have a presentation consistent with the diagnosis of type 2 autoimmune pancreatitis (AIP). AIP is a group of rare heterogeneous diseases that are challenging to diagnose. There are 2 types of AIP. Type 1 disease is the more common worldwide than type 2 AIP. While type 1 AIP is associated with IgG4-positive antibodies, type 2 AIP is IgG4 antibody negative. Both types of AIP are responsive to corticosteroid treatment. Although type 1 AIP has more extrapancreatic manifestations and more commonly relapses, this is a case of a patient with type 2 AIP with inflammatory bowel disease and relapsing course.

scholarly journals Management of autoimmune pancreatitis

2020 ◽  
Vol 48 (3) ◽  
pp. 13-17
Author(s):  
V. S. Akshintala ◽  
V. K. Singh
Keyword(s):  
Autoimmune Pancreatitis ◽  
Pancreatic Disease ◽  
Lymphoplasmacytic Sclerosing Pancreatitis ◽  
Sclerosing Pancreatitis ◽  
Histological Characteristics ◽  
Igg4 Level

The article contains modern data on such a chronic pancreatic disease as autoimmune pancreatitis (AIP). Statistical data on the prevalence and incidence of AIP are presented, the current international classification of this pathology is considered, the main features of type 1 AIP (lymphoplasmacytic sclerosing pancreatitis, LPSP) and type 2 AIP (idiopathic duct centric pancreatitis, IDCP) are put forward. The clinical manifestation of these types is different: obstructive jaundice develops more often in patients with LPSP, while patients with IDCP mostly have acute pancreatitis. The presence and variety of extrapancreatic manifestations of the disease with extremely frequent concomitant development of ulcerative colitis is emphasized. The features of serological diagnosis of AIP and the role of IgG4 level in determining the type of disease are considered. The proper techniques of AIP instrumental diagnostics are listed, the typical changes detected during computed tomography and the distinctive histological characteristics of LPSP and IDCP are considered. The feasibility of a differential diagnosis between AIP and pancreatic cancer is indicated by analyzing the results of serological, imaging, and histological studies. The algorithm of management of AIP patients depending on the type of disease is described, as well as the tactics of prescribing corticosteroids, immunomodulators upon LPSP and IDCP. Immunomodulators of choice (mycophenolate mofetil, azathioprine) are indicated, and rituximab administration features are considered. Possible signs of AI recurrence are listed (IgG4-sclerosing cholangitis, high IgE level).

scholarly journals Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

2019 ◽  
Vol 21 (1) ◽  
pp. 257 ◽  
Author(s):  
Hiroyuki Matsubayashi ◽  
Hirotoshi Ishiwatari ◽  
Kenichiro Imai ◽  
Yoshihiro Kishida ◽  
Sayo Ito ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Critical Role ◽  
Steroid Treatment ◽  
Clinical Images ◽  
Corticosteroid Administration ◽  
Steroid Response ◽  
Sclerosing Pancreatitis

Autoimmune pancreatitis (AIP), a unique subtype of pancreatitis, is often accompanied by systemic inflammatory disorders. AIP is classified into two distinct subtypes on the basis of the histological subtype: immunoglobulin G4 (IgG4)-related lymphoplasmacytic sclerosing pancreatitis (type 1) and idiopathic duct-centric pancreatitis (type 2). Type 1 AIP is often accompanied by systemic lesions, biliary strictures, hepatic inflammatory pseudotumors, interstitial pneumonia and nephritis, dacryoadenitis, and sialadenitis. Type 2 AIP is associated with inflammatory bowel diseases in approximately 30% of cases. Standard therapy for AIP is oral corticosteroid administration. Steroid treatment is generally indicated for symptomatic cases and is exceptionally applied for cases with diagnostic difficulty (diagnostic steroid trial) after a negative workup for malignancy. More than 90% of patients respond to steroid treatment within 1 month, and most within 2 weeks. The steroid response can be confirmed on clinical images (computed tomography, ultrasonography, endoscopic ultrasonography, magnetic resonance imaging, and 18F-fluorodeoxyglucose-positron emission tomography). Hence, the steroid response is included as an optional diagnostic item of AIP. Steroid treatment results in normalization of serological markers, including IgG4. Short- and long-term corticosteroid treatment may induce adverse events, including chronic glycometabolism, obesity, an immunocompromised status against infection, cataracts, glaucoma, osteoporosis, and myopathy. AIP is common in old age and is often associated with diabetes mellitus (33–78%). Thus, there is an argument for corticosteroid therapy in diabetes patients with no symptoms. With low-dose steroid treatment or treatment withdrawal, there is a high incidence of AIP recurrence (24–52%). Therefore, there is a need for long-term steroid maintenance therapy and/or steroid-sparing agents (immunomodulators and rituximab). Corticosteroids play a critical role in the diagnosis and treatment of AIP.

scholarly journals Pancreatitis autoinmune. Serie de casos y actualización bibliográfica

2021 ◽  
Vol 51 (3) ◽  
Author(s):  
Analia Verónica Pasqua ◽  
Juan Carlos Spina ◽  
Eduardo Mullen
Keyword(s):  
Autoimmune Pancreatitis ◽  
Case Reports ◽  
Pancreatic Disease ◽  
Histological Evaluation ◽  
First Case ◽  
Type 1 Autoimmune Pancreatitis ◽  
Pancreatic Disorders ◽  
The 1960S

The concept and diagnostic criteria for autoimmune pancreatitis have evolved since the first case reports published in the 1960s. The advances in knowledge during the past two decades have resulted in the recognition of several pancreatic conditions that show, on histological evaluation, variable combinations of inflammation and fibrosis. In fact, autoimmune pancreatitis comprises two distinct diseases: type 1 and type 2. Type 2 autoimmune pancreatitis is a particular pancreatic disease about which there is limited data; it differs from the ‘classic’ IgG4-related type 1 autoimmune pancreatitis in terms of epidemiology, pathological characteristics and natural evolution. There are patients with complex pancreatic conditions where an autoimmune component and good response to steroids are demonstrated, but they do not meet the suggested criteria for the diagnosis of type 1 or type 2 autoimmune pancreatitis. Although the term steroid-responsive pancreatitis may be interpreted as a synonym to type 1 and 2 autoimmune pancreatitis, these are not the only pancreatic conditions that improve with this type of treatment. Here, we present three clinical cases and a literature review related to the diagnosis and treatment of autoimmune pancreatitis and other less frequent pancreatic disorders that present a good response to steroid therapy.

Recent advances in autoimmune pancreatitis: type 1 and type 2

Gut ◽  
2013 ◽  
Vol 62 (9) ◽  
pp. 1373-1380 ◽  
Author(s):  
Terumi Kamisawa ◽  
Suresh T Chari ◽  
Markus M Lerch ◽  
Myung-Hwan Kim ◽  
Thomas M Gress ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Recent Advances

Autoimmune Pancreatitis

2018 ◽  
Author(s):  
Allison L Yang ◽  
Julia McNabb-Baltar
Keyword(s):  
Chronic Pancreatitis ◽  
Obstructive Jaundice ◽  
Autoimmune Pancreatitis ◽  
Pancreatic Insufficiency ◽  
Imaging Findings ◽  
Laboratory Findings ◽  
Related Disease

Autoimmune pancreatitis (AIP) is a subcategory of chronic pancreatitis that is highly responsive to steroids. The term was first proposed in 1995 by Yoshida and colleagues, and since its discovery, the diagnosis of AIP has dramatically increased. AIP is a chronic fibroinflammatory disease characterized by lymphoplasmacytic infiltrates and fibrosis on histology. There are two distinct subtypes: type 1 AIP is the pancreatic manifestation of a systemic serum immunoglobulin G subtype 4–related disease (IgG4-RD) and type 2 AIP is described clinically as idiopathic duct-centric pancreatitis and has no association with IgG4. Clinically, AIP presents most commonly as obstructive jaundice in type 1 AIP and can present as acute pancreatitis in type 2 AIP. The diagnostic criteria include histology, imaging findings, and responsiveness to steroids as well as laboratory findings and other organ involvement. The mainstay of treatment is steroid therapy, with immunomodulators such as rituximab used for maintenance or relapsing disease. Long-term complications of AIP include pancreatic insufficiency and are often associated with relapsing disease. This review contains 45 references, 1 figure, and 2 tables. Key Words: autoimmune pancreatitis, chronic pancreatitis, EUS-guided biopsy, IgG4, immunomodulatory, obstructive jaundice, pancreas mass, steroid

Surgery in autoimmune pancreatitis

2021 ◽  
Author(s):  
Sara Nikolic ◽  
Poya Ghorbani ◽  
Raffaella Pozzi Mucelli ◽  
Sam Ghazi ◽  
Francisco Baldaque- Silva ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
University Hospital ◽  
Premalignant Lesions ◽  
Single Centre ◽  
Age At Surgery ◽  
Diagnostic Work ◽  
Work Up

Introduction: Autoimmune pancreatitis (AIP) is a disease that may mimic malignant pancreatic lesions both in terms of symptomatology and imaging appearance. The aim of the present study is to analyse experiences of surgery in patients with AIP in one of the largest European cohorts. Methods: We performed a single-centre retrospective study of patients diagnosed with AIP at the Department of Abdominal Diseases at Karolinska University Hospital in Stockholm, Sweden, between January 2001 and October 2020. Results: There were 159 patients diagnosed with AIP, and among them 35 (22.0%) patients had surgery: 20 (57.1%) males and 15 (42.9%) females; average age at surgery was 59 years (range 37-81). Follow-up period after surgery was 67 months (range 1-235). AIP type 1 was diagnosed in 28 (80%) patients and AIP type 2 in 7 (20%) patients. Malignant and premalignant lesions were diagnosed in 8 (22.9%) patients for whom AIP was not the primary differential diagnosis but, in all cases, it was described as a simultaneous finding and recorded in retrospective analysis in histological reports of surgical specimens. Conclusions: Diagnosis of AIP is not always straightforward, and, in some cases, it is not easy to differentiate it from the malignancy. Surgery is generally not indicated for AIP but might be considered in patients when suspicion of malignant/premalignant lesions cannot be excluded after complete diagnostic work-up.

Preoperative magnetic resonance imaging in Type 2 trigeminal neuralgia

2010 ◽  
Vol 113 (3) ◽  
pp. 511-515 ◽  
Author(s):  
Andrew C. Zacest ◽  
Stephen T. Magill ◽  
Jonathan Miller ◽  
Kim J. Burchiel
Keyword(s):  
High Resolution ◽  
Mr Imaging ◽  
Facial Pain ◽  
Neurovascular Compression ◽  
Imaging Reconstruction ◽  
Group A ◽  
Group B ◽  
3D Mr Imaging

Object Trigeminal neuralgia (TN) is a neuropathic pain syndrome that is often associated with neurovascular compression of the trigeminal nerve and may be effectively treated with microvascular decompression (MVD). The authors used high-resolution MR imaging with 3D reconstruction in patients with constant facial pain (Type 2 TN) to determine the presence/absence of neurovascular compression and thus a potential MVD benefit. They retrospectively contacted patients to evaluate outcome. Methods All patients who reported spontaneous onset of constant facial pain (Type 2 TN), which occurred at least 50% of the time, who had undergone high-resolution 3-T MR imaging with 3D reconstruction were retrospectively selected for this study. Clinical history, facial pain questionnaire data, physical examination findings, and results from 3-T 3D MR imaging reconstruction were recorded for all patients. Intraoperative findings and clinical pain outcome were recorded for all patients who underwent MVD. Results Data obtained in 27 patients were assessed. On the basis of history and 3D MR imaging reconstruction findings, 13 patients were selected for MVD (Group A) and 14 underwent conservative treatment (Group B). Typical or suspected artery- or vein-induced neurovascular compression was predicted preoperatively in 100% of Group A patients and in 0% of Group B patients. At the time of MVD, definitive neurovascular compression was confirmed in 11 (84.6%) of 13 Group A patients. Following MVD, facial pain was completely relieved in 3 (23%), improved in 7 (53.8%), and no better in 3 (23%) of 13 Group A patients. A history of episodic (Type 1 TN) pain at any time was reported in 100 and 50% of Group A and Group B patients, respectively. A Type 1 TN pain component was reportedly improved/relieved in all Group A patients, but the Type 2 TN pain component was improved in only 7 (53.8%) of 13 patients. The mean postoperative follow-up duration was 13 months. Conclusions High-resolution 3D MR imaging reconstruction in patients with constant facial pain (Type 2 TN) can help determine the presence/absence of neurovascular compression. Surgical selection based on both clinical and radiological criteria has the potential to improve surgical outcome in patients with Type 2 TN who may potentially benefit from MVD. However, even in such selected patients, pain relief is likely to be incomplete.

Autoimmune pancreatitis complicated with inflammatory bowel disease and comparative study of type 1 and type 2 autoimmune pancreatitis

2014 ◽  
Vol 50 (7) ◽  
pp. 805-815 ◽  
Author(s):  
Shigeyuki Kawa ◽  
◽  
Kazuichi Okazaki ◽  
Kenji Notohara ◽  
Mamoru Watanabe ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Comparative Study ◽  
Autoimmune Pancreatitis ◽  
Bowel Disease ◽  
Inflammatory Bowel
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