Treatment approaches to IgG4-related systemic disease.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Rheumatology Unit, Massachusetts General Hospital, Boston, MA 02114, USA. akhosroshahi@partners.org
Arezou
A
Khosroshahi
John H
JH
Stone
D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology*; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010195:Pancreatitis / Q000188:drug therapy / Q000276:immunology; D011379:Prognosis; D011719:Pyrazines / Q000627:therapeutic use; D012008:Recurrence; D000069283:Rituximab
D058846:Antibodies, Monoclonal, Murine-Derived; D001897:Boronic Acids; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D011719:Pyrazines; D000069283:Rituximab; D000069286:Bortezomib