E-ISSN 1305-3612
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 Diagnostic Radiology Service, Singapore General Hospital, Outram Road, Singapore  
Diagn Interv Radiol ; : -

Abstract

Hamano et al. first described the association between immunoglobin IgG4 and autoimmune pancreatitis in 2001. Since then many previously established diseases have been included within the ambit of this entity and the list continues to grow. These are fibrosclerotic diseases that may demonstrate synchronous or metachronus multi-organ involvement. A diagnostic criteria have been proposed involving (1) serum IgG4 level elevated beyond 135 mg, (2) IgG4 and Ig+ ratio > 40% and > 10 IgG4 per high power field of biopsy sample and (3) constellation of imaging features which span involve include a variety of organ systems. We present a pictorial essay demonstrating the spectrum of imaging findings which include, dacryosialoadenitis, variety of renal lesions, tumefactive thickening of the extra-ocular muscles and orbital nerve, sclerosing cholangitis and type I pancreatitis. Hence, imaging plays an important role in diagnosis, screening for multi-organ involvement and follow up of the disease.

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