Diagnostic and Interventional Radiology
General Radiology - Pictorial Essay

The pancreatic and extrapancreatic manifestations of IgG4-related disease

1.

Department of Diagnostic Radiology, Ng Teng Fong General Hospital, Jurong East, Singapore

2.

Department of Diagnostic Radiology, Ng Teng Fong General Hospital, Jurong East, Singapore

3.

Diagnostic Radiology Service, Singapore General Hospital, Outram Road, Singapore

Diagn Interv Radiol 2018; 24: 83-88
DOI: 10.5152/dir.2018.14319
Read: 719 Downloads: 229 Published: 03 September 2019

Abstract

 

The association between immunoglobulin IgG4 and autoimmune pancreatitis was first shown in 2001. Since then many previously established fibrosclerotic diseases demonstrating synchronous or metachronous multiorgan involvement have been included within the ambit of IgG4-related disease. Diagnostic criteria have been proposed involving 1) serum IgG4 level elevated beyond 135 mg/dL, 2) IgG4+ to IgG+ plasma cell ratio >40% and >10 IgG4+ cells per high power field of biopsy sample and 3) a constellation of imaging features which involve a variety of organ systems. We present a pictorial essay demonstrating the spectrum of imaging findings for IgG4-related disease, including dacryosialadenitis, variety of renal lesions, tumefactive thickening of the extraocular muscles and orbital nerve, sclerosing cholangitis, and type I pancreatitis. Imaging plays an important role in diagnosis, screening for multiorgan involvement, and follow-up of the disease.

 

You may cite this article as: Sangha Brar JS, Gupta S, Haja Mohideen SM, Liauw L, Lath N. The pancreatic and extrapancreatic manifestations of IgG4-related disease. Diagn Interv Radiol 2018; 24:83–88.

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