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.

<jats:p>IgG4-related disease (IgG4-RD) is an increasingly recognised inflammatory and fibrosing condition that commonly shows multisystem involvement. The disease may mimic malignancy and other inflammatory or immune-mediated disorders, but usually has a good response to corticosteroid therapy, underlining the requirement for recognition of the condition. Accurate diagnosis requires careful interpretation of varying combinations of serum IgG4 levels, radiological features and characteristic histopathological appearances within an appropriate clinical setting. The presence of ‘classical’ histopathological features together with an elevated tissue IgG4+ plasma cell count and IgG4 to IgG ratio is often diagnostic and at the very least can strongly support a clinicopathological diagnosis of IgG4-RD. The authors describe the most recent diagnostic criteria for IgG4-RD, especially the histopathological features. The authors then discuss the merits of examining tissues that may be more easily obtainable than those that commonly show the ‘classical’ histopathological features, but within which not all of these ‘diagnostic’ features may be present. The authors conclude that while a combination of ‘classical’ histopathological features and an elevated tissue IgG4+ plasma cell count is the gold standard for diagnosis, examination of tissues that show some but not all of these features can, in the appropriate context, provide useful supporting evidence for a clinicopathological diagnosis of IgG4-RD.</jats:p>

Original publication




Journal article


Journal of Clinical Pathology



Publication Date





963 - 969