IgG4-связанное заболевание с вовлечением тканей глазницы у пятилетнего ребенка

Abstract

Case study IgG4-related disease involving the tissues of the orbit in a five-year-old child Yu.V. Kotova, E.E. Grishina, E.I. Kondratieva, G.I. Fokanova, I.M. Tarabanko Moscow Regional Research Institute of Childhood, Mytishchi, Moscow Region, Russian Federation M.F. Vladimirsky Moscow Regional Research Institute, Moscow, Russian Federation Purpose. To demonstrate a rare clinical case of IgG4-related disease in the practice of a rheumatologist, a pediatrician and an ophthalmologist. Material and methods. A 5-year-old child had a lesion in the left orbit, manifested by left-sided exophthalmos, medial displacement of the left eye, and limited outward eye mobility. He underwent an orbitotomy with histological and immunohistochemical examination of the biopsy specimen in the ophthalmology department of M.F. Vladimirsky Moscow Regional Research Institute. A diagnosis of IgG4-related disease involving the left orbital tissue was made. Results. In the rheumatology department of the Moscow Regional Research Clinical Institute of Childhood, the boy received pulse therapy with glucocorticoids. When trying to reduce the dose of glucocorticoids, exophthalmos and swelling of the eyelids on the left increased again. Cyclophosphamide therapy was ineffective. In addition to oral Prednisolone, Rituximab, a drug from the group of synthetic chimeric monoclonal antibodies, was prescribed. A stabilization of the process in the left orbit and an improvement in the general condition of the child ocurred. The duration of remission is 2 years. Discussion. IgG4-related disease is characterized by lymphoplasmacytic infiltration of tissues with a predominance of IgG4-positive cells and subsequent formation of fibrosis. Ophthalmological manifestations of IgG4-related disease are most often characterized by damage to the tissues of the orbit and lacrimal gland. Usually develops in middle-aged people; it is rare in pediatric practice. Conclusion. Patients with IgG4-related disease can be encountered in the practice of a rheumatologist, pediatrician and ophthalmologist. If diagnosis and treatment are not urgent, the disease is prone to progression, which leads to disability of the patient. The main treatment method is long-term use of glucocorticoids. In case of steroid resistance or steroid dependence, early use of immunosuppressive drugs or monoclonal antibodies could be considered. Key words: immunoglobulin G4-related disease, autoimmune orbital myositis, pediatric rheumatology, immunosuppressive therapy