IgG4-related disease: the state of art

In 2003, the Japanese authors reported a case series of patients with autoimmune pancreatitis and systemic manifestations showing tissue infiltration with IgG4-positive cells and fibrosis. The first nomenclature of IgG4-related diseases that was developed in 2012 highlights their protean clinical picture, which, along with the absence of specific laboratory tests, underlies diagnostic challenges. In recent years, multiple studies of IgG4-related disease were undertaken and gave rise to classification criteria that have been developed by the American College of Rheumatology and The European League Against Rheumatism. The review of the literature highlights the current management of patients with IgG4-related disease.

Key words

IgG4-related disease, autoimmune pancreatitis, Mikulicz syndrome