IgG4-Related Disease and Successful Treatment with Rituximab in a Three-Year-Old Boy

Abstract

IgG4-Related Disease (IgG4-RD) is a rare inflammatory disease, which may affect all organs and lead to severe fibrosis and organ damage. Mainly described in Japanese or Asian adults, case reports on children-variously affected by the disease-are increasing worldwide. While glucocorticoids are broadly used as first line treatment, several cases require second line options due to relapse or resistance to first line treatment. We report a boy who was diagnosed with IgG4-related disease at the age of three years. He presented an IgG4-associated tumorous lesion in the upper lobe of the left lung, pleural and pericardial effusion and enlarged abdominal lymph nodes. Therapy with surgery and glucocorticoids followed by rituximab treatment led to continuous complete remission 27 months after diagnosis of IgG4-RD.