PECULIARITIES OF THE COURSE OF IgG4-RELATED DISEASE IN CHILDREN

Purpose of this research was to summarize data on the features of the course of IgG4-related disease (IgG4-RD) in children in a series of clinical observations. Materials and methods used: a single-center observational study included four pediatric patients with both clinically and morphologically confirmed IgG4-RD. Age of onset, duration of the disease prior to diagnosis, clinical, instrumental, laboratory and morphological studies were analyzed at the time of onset and during follow-up. The duration of follow-up ranged from 6 months to 7 years. The disease manifested with orbital pseudotumor in two patients, with diabetes insipidus in a single patient and with fever, signs of high inflammatory and immunological activity in another patient. The period for diagnosis was 3 to 12 months. The diagnosis was based on a biopsy of orbital tissue with detection of signs of fibrosis, obliterating phlebitis, and subsequent immunohistochemical testing for the presence of IgG4. Three children received basic therapy with steroids and cytostatics, a single child had received cytostatics solely. A clinical effect was achieved during therapy: no progression of the disease was recorded in all patients according to instrumental observation.