The Netherlands journal of medicine
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IgG4-related disease (IgG4-RD) is an emerging systemic inflammatory disease involving nearly all organs eventually leading to fibrosis. Prompt and adequate treatment to prevent irreversible organ damage is therefore pivotal. To evaluate the treatment outcomes, we studied a well-defined cohort of patients with IgG4-RD. ⋯ Glucocorticoids and rituximab induce substantial responses as well as primary surgical intervention and radiotherapy, while the efficacy of DMARDs is limited. Based on the few data available, hydroxychloroquine, infliximab and thalidomide may be promising treatment options for second or third line strategies.