Systemic manifestations of IgG4-associated disease

Abstract

The article presents general data on IgG4-associated disease, as well as its classification and universal diagnostic criteria. Attention is focused on the possibility of multi-organ lesions, which complicates diagnosis and treatment. A clinical case of an IgG4-associated disease involving the retroperitoneal space (retroperitoneal fibrosis), lungs (bilateral interstitial pneumonia, diffuse and focal fibroid lung), intestines (chronic colitis) in a middle-aged man is described. Sequential administration of glucocorticoids and monoclonal antibodies made it possible to bring a patient into remission, however, the recurrence of the disease required the resumption of hormone therapy at a reduced dose with a positive effect.