Arezou Khosroshahi1, John H Stone. 1. Rheumatology Unit, Massachusetts General Hospital, Boston, MA 02114, USA. akhosroshahi@partners.org
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.
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.
Authors: Zachary S Wallace; Hamid Mattoo; Vinay S Mahajan; Maria Kulikova; Leo Lu; Vikram Deshpande; Hyon K Choi; Shiv Pillai; John H Stone Journal: Rheumatology (Oxford) Date: 2016-02-16 Impact factor: 7.580
Authors: Yassaman Raissian; Samih H Nasr; Christopher P Larsen; Robert B Colvin; Thomas C Smyrk; Naoki Takahashi; Ami Bhalodia; Aliyah R Sohani; Lizhi Zhang; Suresh Chari; Sanjeev Sethi; Mary E Fidler; Lynn D Cornell Journal: J Am Soc Nephrol Date: 2011-06-30 Impact factor: 10.121