Abstract
IgG4-related disease is a chronic inflammatory fibrosing disease of unknown etiology, characterized by the presence of volumetric lesions that can clinically simulate malignant tumors, a pronounced IgG4-positive lymphoplasmacytic infiltrate, and an increase in the level of IgG4 in the blood serum. A special form of the disease is IgG4-related ophthalmopathy, which requires differential diagnosis with inflammatory pseudotumor, lymphoma and granulomatous polyangiitis. 7 clinical cases of IgG4-related ophthalmopathy are presented. It has been shown that follicle-like structures with the structure of a lymph node are formed in the tissues of the orbit. Along with a large number of cells in the inflammatory infiltrate expressing CD138, IgG and IgG4, there are CD8+ and CD68+ cells. IgG4-related ophthalmopathy is a rare manifestation of IgG4-related disease. Its morphogenesis involves not only IgG, IgG4 and CD138 positive plasma cells, which are diagnostic. CD8 and CD68- positive cells are involved too. They persisted in large quantity in the lymphohistiocytic infiltrate. The study of the lymphocyte population can help in revealing the pathogenesis and morphogenesis of this rare disease.
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