Treatment Approaches to IgG4-related Systemic Disease

Arezou Khosroshahi; John H. Stone


Curr Opin Rheumatol. 2011;23(1):67-71. 

In This Article

Abstract and Introduction


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.


The response to treatment in IgG4-related systemic disease (IgG4-RSD) is swift and often dramatic, provided that therapy is initiated at a time before the fibroinflammatory process becomes densely sclerotic. Indeed, one of the characteristics that distinguishes autoimmune pancreatitis (AIP) and IgG4-associated cholangitis from their principal mimickers is the responsiveness of the IgG4-RSD conditions to glucocorticoid treatment. Treatment responsiveness has been used in the Korean and Mayo Clinic diagnostic criteria for AIP.[1,2] Prompt improvement following the initiation of glucocorticoid treatment is often interpreted as a useful diagnostic sign for cases in which diagnostic certainty has been elusive.

There have been no randomized controlled studies for treatment of either IgG4-RSD or AIP. Issues pertaining to the treatment of AIP have been considered in detail, but the best data supporting all treatment approaches are based on retrospective, observational studies that have included relatively few patients. In addition, except for case reports, there exist virtually no data about the treatment of extrapancreatic IgG4-RSD. Even studies of AIP that have included patients with extrapancreatic disease (e.g. sialadenitis, lacrimal gland disease, retroperitoneal fibrosis) have not commented in a systematic way on the outcomes of treatment in nonpancreatic organs.

Here, we review the current status and recent advances in the treatment approach to AIP and IgG4-RSD.


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