Cookies on this website

We use cookies to ensure that we give you the best experience on our website. If you click 'Accept all cookies' we'll assume that you are happy to receive all cookies and you won't see this message again. If you click 'Reject all non-essential cookies' only necessary cookies providing core functionality such as security, network management, and accessibility will be enabled. Click 'Find out more' for information on how to change your cookie settings.

© 2019 Immunoglobulin (Ig) G4-related disease is an immune-mediated fibro-inflammatory condition that can involve virtually every organ system. Our knowledge and understanding of this condition has expanded rapidly over the last decade, with insights into adaptive and innate immunological mechanisms, identification of novel risk factors, definition of disease subsets based on organ distribution, discovery of circulating biomarkers that correlate with disease activity and relapse, and novel treatment approaches. However, many challenges remain. The diagnostic work-up remains complex, requiring a combination of clinical examination, serology, imaging and histological evaluation with cross-speciality input. This requires the exclusion of a broad variety of differential diagnoses. Treatment often dampens inflammation, although relapse remains common on tapering or discontinuation of best available immunosuppression. Progressive fibrosclerotic disease and an increased risk of malignancy are recognized outcomes.

Original publication

DOI

10.1016/j.mpmed.2019.09.005

Type

Journal article

Journal

Medicine (United Kingdom)

Publication Date

01/12/2019

Volume

47

Pages

804 - 807