Cross-sectional pictorial review of IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder characterized by the infiltration of one or more organs with IgG4-positive plasma cells resulting in inflammatory lesions and fibrosis. Although the pancreas is the most commonly affected organ, involvement of extrapancreatic orga...

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Bibliographic Details
Published in:British journal of radiology 2019-11, Vol.92 (1103), p.20190448-20190448
Main Authors: Kurowecki, Darya, Patlas, Michael N, Haider, Ehsan A, Alabousi, Abdullah
Format: Article
Language:English
Subjects:
Adult
Cardiovascular Diseases - diagnosis
Cardiovascular Diseases - etiology
Dacryocystitis - diagnosis
Dacryocystitis - etiology
Female
Gallbladder Diseases - diagnosis
Gallbladder Diseases - etiology
Humans
Immunoglobulin G4-Related Disease - complications
Immunoglobulin G4-Related Disease - diagnosis
Kidney Diseases - diagnosis
Kidney Diseases - etiology
Lung Diseases - diagnosis
Lung Diseases - etiology
Magnetic Resonance Imaging
Male
Mastitis - diagnosis
Mastitis - etiology
Middle Aged
Multidetector Computed Tomography
Orbital Pseudotumor - diagnosis
Orbital Pseudotumor - etiology
Pancreatic Diseases - diagnosis
Pancreatic Diseases - etiology
Pictorial Review
Retroperitoneal Fibrosis - diagnosis
Retroperitoneal Fibrosis - etiology
Sialadenitis - diagnosis
Sialadenitis - etiology
Ultrasonography
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Description
Summary:Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder characterized by the infiltration of one or more organs with IgG4-positive plasma cells resulting in inflammatory lesions and fibrosis. Although the pancreas is the most commonly affected organ, involvement of extrapancreatic organs is an increasingly recognized manifestation of the disease. Patients may be asymptomatic and serum IgG4 concentrations may be elevated or normal. Treatment consists of glucocorticosteroid treatment, with excellent response. A definitive diagnosis requires histopathology with imaging playing a key role in avoiding treatment delays. This pictorial review will focus on the most current knowledge regarding IgG4-RD including its common and less common manifestations and the roles of multidetector CT, MRI and ultrasound in the diagnosis and management of suspected IgG4-RD. Knowledge of the varied imaging findings of this multi systemic disease is essential for radiologists to avoid misdiagnosis and assist with timely and effective treatment.
ISSN:0007-1285
1748-880X
DOI:10.1259/bjr.20190448