Аннтотация

© 2018, Pediatria Ltd.. All rights reserved. The article presents the case of IgG4-associated disease (IgG4-AD) with orbit lesion in a 5-year-old girl. IgG4-AD is a chronic immune-mediated disease of unknown etiology characterized by tumor-like infiltration of various organs tissues with plasma cells expressing IgG4 and eosinophilic infiltration followed by the development of fibrous fibrosis and obliterating phlebitis, and with increase of IgG4 levels in serum in a number of patients. The majority of clinical observations are associated with mature and older patients. In children, especially young, IgG4-AD is rare, so the description of each case is interesting for pediatricians and various specialists working with pediatric patients.