To review radiological features of IgG4-Related Disease (IgG4-RD) in the abdomen and pelvis and their correlation with physiopathological and clinical features.
To evaluate the utility of multimodality cross-sectional imaging in its diagnosis.
To highlight the imaging clues for an appropriate differential diagnosis.
IgG4-related disease (IgG4-RD) is a multisystemic and increasingly diagnosed immunemediated fibroinflammatory condition characterized by diffuse or focal tumor lesions with a dense lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells associated with obliterative phlebitis and a variable degree of fibrosis.
Elevated IgG4 serum levels are found in 60-70% of patients,
but they are not pathogenic and only represent a down-regulatory response of the immune system.
IgG4-RD mostly affects men older than 50 years-old.
The disease is also increasingly recognized to occur in children.
It can synchronously...
Findings and procedure details
IgG4-related Pancreatitis (Fig. 1,Fig. 2)
IgG4-related pancreatitis is the most common intrabdominal manifestation of the IgG4-RD and it accounts for 5-11% of chronic pancreatitis.
Patients present with similar symptoms of pancreatic adenocarcinoma or chronic pancreatitis,
such as mild abdominal pain,
and new-onset diabetes.
Radiological features are key clues in the differential diagnosis along with involving of extrapancreatic organs and responsiveness to glucocorticoids.
IgG4-related pancreatitis presents as a diffuse enlargement with loss of
pancreatic clefts (“sausage-shaped” sign) or segmental enlargement...
IgG4-RSD is a multisystemic fibroinflammatory condition that mimics many neoplastic,
infectious and inflammatory disorders.
Radiologists should be able to recognize key radiological features and frequent multiorgan involvement of the disease to establish an accurate and timely diagnosis in combination with clinical history and serologic markers and to avoid unnecessary invasive procedures.
José Miguel Escudero-Fernández
Department of Abdominal Imaging
Hospital Universitari Materno-Infantil Vall d’Hebron,
Institut català de la Salut.
Passeig de la Vall d’Hebron 119-129 08035 Barcelona Spain
A new clinicopathological entity of IgG4-related autoimmune disease.
The spectrum of IgG4-related disease in the abdomen and pelvis.
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Focal autoimmune pancreatitis: radiological characteristics help to distinguish from pancreatic cancer.
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