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Paper 3
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
A 42 year old woman with a history of Sjögren syndrome presents with acute abdominal pain. On examination she is tender with guarding in the epigastric region. Blood tests show highly elevated serum amylase level. She undergoes a CT abdomen pelvis with contrast. This shows a diffusely enlarged pancreas with a surrounding hypoattenuating rim. There is minimal peripancreatic stranding and no pseudocyst formation or necrosis. An autoimmune pancreatitis is considered to be most likely clinically.
IVIM MRI of the Pancreas
Published in Denis Le Bihan, Mami Iima, Christian Federau, Eric E. Sigmund, Intravoxel Incoherent Motion (IVIM) MRI, 2018
Miriam Klauß, Philipp Mayer, Bram Stieltjes
Pancreatic ductal adenocarcinomas (PDACs), which are the most frequent malignant pancreatic tumors, often appear hypovascular compared to the healthy pancreatic parenchyma in conventional contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI). PDAC is the fourth-most-frequent cause of death from cancer worldwide. The prognosis is poor at the time of diagnosis and is usually made at late stages of the disease due to its unspecific clinical signs and symptoms. In contrast, pancreatic neuroendocrine tumors (PNETs) represent the second-most- common solid pancreatic malignancy and characteristically appear hypervascular compared to normal pancreatic tissue in contrast- enhanced cross-sectional imaging. Chronic pancreatitis and autoimmune pancreatitis are benign pancreatic diseases, which can be difficult to differentiate from malignant adenocarcinomas because of the at-times mass-forming aspect and the often hypovascular appearance compared to the healthy pancreatic parenchyma in conventional CT and MRI.
The pancreas
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
Autoimmune pancreatitis has been described relatively recently. Features include diffuse enlargement of the pancreas, and diffuse and irregular narrowing of the main pancreatic duct. It may occur in association with other autoimmune diseases, as a multisystem disorder, or may affect the pancreas alone. There may be changes in the biliary tree (autoimmune cholangiopathy) as well. The changes may be confused with neoplasia. Autoantibodies may be present, and levels of the immunoglobulin subtype IgG4 are elevated.
IgG4-related disease presenting as a paraneoplastic syndrome: report of two cases and literature review
Published in Modern Rheumatology Case Reports, 2021
Mitsuteru Akahoshi, Yojiro Arinobu, Yusuke Kashiwado, Aya Omoto, Masahiro Ayano, Hiroki Mitoma, Yasutaka Kimoto, Nobuyuki Ono, Takahiko Horiuchi, Hiroaki Niiro
We describe two cases in which malignant tumours were observed after the onset of IgG4-RD, and surgical treatment resulted in spontaneous regression of IgG4-RD itself. A previous report examining the association between autoimmune pancreatitis (AIP) and cancer suggests that AIP may have developed as a paraneoplastic syndrome in some patients, as there have been no AIP relapse after successful treatment of coexisting cancer [4]. We also conducted a literature search in the PubMed database to identify the peer-reviewed cases associated with IgG4-RD and paraneoplastic syndrome in the literature published by January 10, 2021. The search strategies included the following terms without language restriction: (“IgG4-related disease" OR “autoimmune pancreatitis” OR “Mikulicz's disease”) AND “paraneoplastic syndrome*”. As a result, two cases of IgG4-RD, considered to be a paraneoplastic syndrome, have been described so far [8,9]. In all four cases, including our two cases, IgG4-RD was diagnosed at the same time as the onset or recurrence of cancer or within 1 year, and treatment for cancers that resulted in regression of IgG4-RD included tumour resection or cancer chemotherapy.
Acute pancreatitis without abdominal pain induced by administration of nivolumab and ipilimumab
Published in Modern Rheumatology Case Reports, 2021
Koichiro Yamamoto, Kosuke Oka, Reina Son, Hiroyuki Honda, Hiroyuki Sakae, Kou Hasegawa, Shigeru Horiguchi, Hironari Kato, Osamu Yamasaki, Fumio Otsuka
ICI-induced pancreatitis has rarely been reported, but it can be fatal [5]. The incidences of ICI-induced pancreatitis shown by a systematic review and meta-analysis were 0.94% in patients treated with a PD-1 inhibitor [95% confidence interval (CI): 0.48–1.40], 3.98% in patients treated with a CTLA-4 inhibitor [95% CI: 2.92–5.05], and 10.60% in patients treated with a PD-1 inhibitor + CTLA-4 inhibitor [95% CI: 7.89–13.32], with significant differences between the groups [6]. The risk ratio (RR) for the all-grade elevation of lipase was significantly higher in patients receiving combination therapy of nivolumab and ipilimumab than in patients receiving nivolumab or ipilimumab treatment alone (RR: 6.43 [95% CI: 1.43–28.99]) [4]. The RR for all-grade amylase elevation was also significantly higher in patients receiving combination therapy (6.08 [95% CI: 1.51–24.44]) [4]. Of particular interest, the risk of pancreatitis after ICI administration (PD-1 and CTLA-4 inhibitors alone or in combination) was not significantly elevated compared to that with control therapy [4]. In that study, the authors concluded that there was a discrepancy between elevated pancreatic enzymes and clinically significant pancreatitis, suggesting that imaging tests were important for detecting immune-related pancreatitis [4]. Thus, they proposed that the criteria for the diagnosis of autoimmune pancreatitis (AIP), which include the five factors of imaging, serology, histology, extrapancreatic involvement, and steroid responsiveness, might be useful for confirming ICI-induced pancreatitis [4].
IgG4-related disease presenting as intradural extramedullary lesion: a case report and review of the literature
Published in British Journal of Neurosurgery, 2019
Kelly J. Bridges, Caitlin H. DeDeaux, Khoi D. Than
IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder involving formation of tumefactive lesions at multiple anatomical sites.1,2 Lesions contain storiform fibrosis and lymphoplasmacytic infiltrate rich in IgG4.1 The disease was originally recognized in the pancreas (called autoimmune pancreatitis) and was not recognized as a systemic condition until 2003, when extra-pancreatic manifestations were identified.2 IgG4-RD can affect several alternative organs as well, often involving the bile ducts, salivary glands, lacrimal gland, mediastinal lymph nodes, retroperitoneum, aorta, lungs, and kidneys.3–5 Lesions may be synchronous or metachronous.6 The thoracic paravertebral space is commonly involved, most frequently in elderly males, in whom computed tomography (CT) will show thickening of soft tissue in the thoracic paravertebral space.7