Abstract
The spectrum of IgG4-related diseases
Highlights
IgG4 In sera of normal individuals there are four IgG isotypes: IgG1, IgG2, IgG3 and IgG4
IgG4-related disease (IgG4-RD) is a newly-described rare syndrome consisting of many disease entities that were previously thought to be unrelated
The majority of patients respond to glucocorticoids, in early stages of disease [1]. Major presentations of this protean condition, which often affects more than one organ, include: type 1 (IgG4-related) autoimmune pancreatitis (AIP), salivary gland disease, which can present as major salivary gland enlargement or as sclerosing sialadenitis
Summary
IgG4 In sera of normal individuals there are four IgG isotypes (subclasses): IgG1, IgG2, IgG3 and IgG4. IgG4-related disease (IgG4-RD) is a newly-described rare syndrome consisting of many disease entities that were previously thought to be unrelated These conditions have the common pathological features of: inflammatory pseudotumours, i.e., swelling of organs (organomegaly) or nodules within an organ; lymphoplasmacytic infiltration of tissues with many IgG4-positive plasma cells; storiform fibrosis (scarring involving cells arranged like a cartwheel on histology). IgG4-related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. It was recognised as a unified entity only 10 years ago. The majority of patients respond to glucocorticoids, in early stages of disease [1] Major presentations of this protean condition, which often affects more than one organ, include: type 1 (IgG4-related) autoimmune pancreatitis (AIP), salivary gland disease, which can present as major salivary gland enlargement or as sclerosing sialadenitis. Autoreactive IgG4 antibodies are observed in IgG4-RD, there is no evidence that they are directly path-
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