Abstract
Abstract. Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder, characterized by a tissue IgG4(+)plasma cells infiltration, leading to fibrosis and destruction of the involved organs. The IgG4-RD scope includes all organs and systems but within the review only the actual diagnostic and therapeutic data concerning disease localisations in the lacrimal and salivary glands, kidneys, retroperitoneum, lungs and aorta and arteries are included because they are also a terrain of a rheumatological pathology – vasculitides, Sjogren’s syndrome, SLE, systemic sclerosis and so on.
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