Systemic disorders with rheumatic manifestations: Edited by John Stone and Arezou KhosroshahiTreatment approaches to IgG4-related systemic diseaseKhosroshahi, Arezou; Stone, John HAuthor Information Rheumatology Unit, Massachusetts General Hospital, Boston, Massachusetts, USA Correspondence to Arezou Khosroshahi, MD, Rheumatology Unit, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA Tel: +1 617 726 2870; e-mail: firstname.lastname@example.org Current Opinion in Rheumatology: January 2011 - Volume 23 - Issue 1 - p 67-71 doi: 10.1097/BOR.0b013e328341a240 Buy Metrics Abstract Purpose of review IgG4-related systemic disease (IgG4-RSD) is a systemic fibroinflammatory condition that can affect any organ system. Prompt recognition and management of this disease process are necessary to prevent sclerosis and permanent organ damage. Here, we review the advances in treatment approaches to IgG4-RSD. Recent findings Most information regarding treatment is derived from retrospective case series of patients with autoimmune pancreatitis (AIP), and follow-up periods have generally been short. A variety of IgG4-RSD presentations respond rapidly to glucocorticoid treatment. Glucocorticoids have become a standard therapy for AIP, but the indications requiring treatment as well as the appropriate starting dose and duration of therapy remain controversial. The importance of maintenance of glucocorticoids following remission induction is debatable. As our knowledge grows regarding other organ manifestations of IgG4-RSD with longer follow-ups, the necessity of steroid-sparing agents to manage frequent relapses becomes clear. Summary The natural history and long-term prognosis of IgG4-RSD are not well understood. Large prospective studies and randomized controlled trials of patients with wide spectrum manifestations of IgG4-RSD are required to support better approaches to treatment. © 2011 Lippincott Williams & Wilkins, Inc.