Поражения глаз при гранулематозе Вегенера

Abstract

Wegener's granulomatosis, also called granulomatosis with polyangiitis, is a primary systemic necrotizing vasculitis of small and medium-sized vessels with the formation of granulomatous inflammation. This is a severe, progressive disease, presumably of autoimmune nature, which without basic treatment – immunosuppressive therapy (suppression of disease activity, remission maintenance and episodic therapy) can lead to the death of the patient. Upper respiratory tract, eye, lung and kidney lesions prevail in the clinical picture of Wegener's granulomatosis. Ocular manifestations often appear at early stages of the disease and are associated with upper respiratory tract and/or middle ear inflammation. Wegener's granulomatosis can affect any segment of the eye, most often the anterior segment and much less often the posterior, and can present in the form of minor and moderate ocular inflammation (scleritis, keratitis, etc.) or severe disorders of all structures and orbits (pseudotumour) leading to low vision and even blindness. Difficulties in the diagnosis of Wegener's granulomatosis arise from a variety of forms and ways of clinical progression at early stages of the disease, when ophthalmic changes are not very specific. In order to establish the correct diagnosis, it is necessary to compare biopsy results (characterized by granulomatous inflammation and vasculitis of small vessels, areas of necrosis) with clinical laboratory assessments. The refractory course of pathological process in the orbit, eye and its appendages, especially if the patient has some atypically occurring pathological processes in the upper respiratory tract and/or sinuses and lungs, should be a warning sign for a doctor to use an interdisciplinary approach for the diagnosis and treatment. Key words: Wegener's granulomatosis, granulomatosis with polyangiitis, systemic necrotizing vasculitis, eye disorder, scleritis, orbital pseudotumour