Morphologic Patterns of Autoimmune Pancreatitis in CT and MRI

Rehnitz, Christoph; Klauß, Miriam; Singer, Reinhard; Ehehalt, Robert; Werner, Jens; Büchler, Markus W.; Kauczor, Hans‐Ulrich; Grenacher, Lars

doi:10.1159/000327708

Cited by 43 publications

(26 citation statements)

References 34 publications

(52 reference statements)

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“…The latter pseudotumoural lesions frequently can have the aforementioned "late enhancement" after intravenous contrast. The latter is best detectable in MRI with an overall better diagnostic outcome compared to CT particularly in the venous and the late phase [30]. (ii) The typical loss of the physiological lobulation of the pancreas and (iii) the typical contrastographic appearance (see above).…”

Section: Manifestations In the Gastrointestinal Tractmentioning

confidence: 99%

IgG4-Related Diseases in the Gastrointestinal Tract: Clinical Presentation, Diagnosis and Treatment Challenges

et al. 2018

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Background: IgG4-related diseases are a rare but an important entity. Due to the variable clinical presentation, this multiorgan disease was attributed to single-organ systems for many years. Also, it often remains a challenge to differentiate between IgG4-related diseases and malignancies. The pathogenesis seems to be a mixture of Th1- and Th2- immune responses, whereas the role of the non-pathogenic IgG4 antibodies is still unclear. Histopathological characteristics are a lymphoplasmacellular infiltrate with IgG4+ plasma cells, a storiform fibrosis and an obliterative phlebitis. This can lead to the functional destruction of every organ affected. In most cases, glucocorticoid treatment leads to remission and is used as maintenance therapy as well. Immune modulatory therapies are employed in case of steroid resistance. However, a majority of patients achieve remission without any therapy. Summary: In this study, we review the current state-of-the-art regarding pathophysiology, diagnostics, organ manifestation and therapeutic approaches. Key Messages: While the diagnosis of IgG4-related diseases is still challenging, there have been significant improvements in diagnostic as well as in therapeutic approaches. This is partially due to a better understanding of the pathophysiology of the disease but also due to improved imaging modalities and novel, more targeted therapies.

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Section: Manifestations In the Gastrointestinal Tractmentioning

confidence: 99%

IgG4-Related Diseases in the Gastrointestinal Tract: Clinical Presentation, Diagnosis and Treatment Challenges

et al. 2018

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“…Typical MRI findings in AIP include hypo-intense signal on T1 weighted images, lower signal intensity in the presence of intense fibrosis or relatively T2 hyper intensity with minimal fibrosis (46,51). MRI also demonstrates the typical capsule-like rim as a hypo-intense rim on both T1 and T2 weighted images and shows a delayed enhancement on dynamic MR study (46).…”

Section: Imagingmentioning

confidence: 99%

Autoimmune pancreatitis

Omiyale¹

2016

Gland Surg.

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Autoimmune pancreatitis (AIP) is a rare, distinct and increasingly recognized form of pancreatitis which has autoimmune features. The international consensus diagnostic criteria (ICDC) for AIP recently described two subtypes; type 1[lymphoplasmacytic sclerosing pancreatitis (LPSP)] and type 2 [idiopathic duct-centric pancreatitis (IDCP) or AIP with granulocytic epithelial lesion (GEL)]. Type 1 is the more common form of the disease worldwide and current understanding suggests that it is a pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD). In contrast, type 2 AIP is a pancreasspecific disease not associated with IgG4 and mostly without the overt extra-pancreatic organ involvement seen in type 1. The pathogenesis of AIP is not completely understood and its clinical presentation is nonspecific. It shares overlapping features with more sinister pathologies such as cancer of the pancreas, which continues to pose a diagnostic challenge for clinicians. The diagnostic criteria requires a variable combination of histopathological, imaging and serological features in the presence of typical extrapancreatic lesions and a predictable response to steroids.

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“…The classic description of autoimmune pancreatitis is of diffuse pancreatic enlargement with a capsule like rim, T1 hypointensity on MRI and delayed phase enhancement on both CT and MRI studies . More recent studies suggest that the late enhancement of involved pancreas is more frequently appreciated at MRI compared with CT examinations and the gland is hypoenhancing in the arterial phase for both modalities . The rim sign is described in 25–40% of cases and is a seen as a periglandular halo comprising an inflammatory infiltrate that is hypodense on CT and hypointense on T1‐ and T2‐weighted images (Figs , ).…”

Section: Autoimmune Pancreatitismentioning

confidence: 99%

Imaging features of immunoglobulin G4‐related disease

2016

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SummaryImmunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that was first recognised as a systemic disease in 2003, when patients with autoimmune pancreatitis were found to have extrapancreatic manifestations. Since 2003, IgG4-RD has been described in a diverse range of other organs including the biliary tree, orbits, lacrimal glands, salivary glands, lungs, kidneys, aorta, retroperitoneum, lymph nodes, pachymeninges, prostate and pituitary gland. The disease frequently occurs in the absence of pancreatic involvement. The imaging manifestations of IgG4-RD are broad and variable depending on the organ involved. The majority of individual organ appearances are non-specific and differentiation between IgG4-RD and potentially more serious conditions is not usually possible based on radiological findings in one organ alone. However, if there are simultaneous findings typical of IgG4-RD in multiple organs then this is a key diagnostic clue and IgG4-RD should be considered as one of the main differentials. This review article examines the spectrum of imaging appearances of IgG4-RD. Increased awareness of the spectrum of radiological appearances of IgG4-RD throughout the body and subsequent consideration of the condition may potentially avoid invasive treatment and lead to more prompt corticosteroid therapy.

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Morphologic Patterns of Autoimmune Pancreatitis in CT and MRI

Cited by 43 publications

References 34 publications

IgG4-Related Diseases in the Gastrointestinal Tract: Clinical Presentation, Diagnosis and Treatment Challenges

IgG4-Related Diseases in the Gastrointestinal Tract: Clinical Presentation, Diagnosis and Treatment Challenges

Autoimmune pancreatitis

Imaging features of immunoglobulin G4‐related disease

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