Abstract
IgG4-related disorders (IgG4-RD) are a new entity of multi-organ disorders that share common fibro-inflammatory histopathologic characteristics and elevated IgG4 levels in serum and tissue. Autoimmune pancreatitis and Mikulicz syndrome are major entities, however many syndromes are part of IgG4-RD including eosinophilic angiocentric fibrosis, fibrosing mediastinitis, hypertrophic pachymeningitis, inflammatory pseudotumour, multifocal fibrosclerosis (commonly affecting the orbits, thyroid gland, retroperitoneum, mediastinum, and other tissues and organs), periaortitis and peri-arteritis, inflammatory aortic aneurysm, retroperitoneal fibrosis (Ormond’s disease), Riedel’s thyroiditis, and sclerosing mesenteritis. In this review we detail the link between IgG4-RD and endocrine disorders and then examine the link between diabetes and IgG4-RD. We study the increased risk of IgG4-RD after using dulaglutide. We concluded that IgG4-RD should be suspected in patients with progressive thyroiditis with mass effect and very high antibody titers. The relationship between IgG4-RD and diabetes is still controversial.
Highlights
In 2001, Hamano et al [1] described elevated levels of IgG4 in the sera of patients presenting with autoimmune pancreatitis
IgG4-related disorders (IgG4-RD) are described as “a fibro-inflammatory condition characterized by a tendency for formation of tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, frequent, but not invariable elevations of serum IgG4 levels, and a swift initial response to glucocorticoids provided that tissue fibrosis has not supervened” [2]
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that presents with painless obstructive jaundice
Summary
In 2001, Hamano et al [1] described elevated levels of IgG4 in the sera of patients presenting with autoimmune pancreatitis. A few years later, IgG4-positive cells were described in other organs of these patients, and the term IgG4-related disorders was suggested. IgG4-RD are described as “a fibro-inflammatory condition characterized by a tendency for formation of tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, frequent, but not invariable elevations of serum IgG4 levels, and a swift initial response to glucocorticoids provided that tissue fibrosis has not supervened” [2].
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