SYSTEMIC DISORDERS WITH RHEUMATIC MANIFESTATIONS: Edited by John Stone and Arezou KhosroshahiThe latest on IgG4-RD a rapidly emerging diseaseCarruthers, Mollie N.; Stone, John H.; Khosroshahi, Arezou Author Information Division of Rheumatology, Department of Medicine, Rheumatology Unit, Allergy and Immunology, Boston, Massachusetts, USA Correspondence to John H. Stone, MD, MPH, Rheumatology Unit, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA. Tel: +1 617 726 3165; fax: +1 617 643 1274; e-mail: [email protected] Current Opinion in Rheumatology: January 2012 - Volume 24 - Issue 1 - p 60-69 doi: 10.1097/BOR.0b013e32834ddb4a Buy Metrics Abstract Purpose of review Provide an update of the developments in IgG4 related disease within the last year in terms of nomenclature, diagnostic criteria and clinical features. Recent findings The number of diseases associated with IgG4 related disease continues to grow. The entities of idiopathic orbital inflammation, idiopathic cervical fibrosis, eosinophilic angiocentric fibrosis, reactive nodular fibrous pseudotumor, sclerosing mesenteritis, and membranous glomerulonephritis should all be added to the list of diseases associated with the IgG4 related disease spectrum. The issues of nomenclature and diagnostic criteria for IgG4 related disease have important recent developments as well. Summary The diagnosis of IgG4 related disease continues to be challenging. Increasing recognition of IgG4 related disease has led to a large body of literature on organ sites of involvement. Understanding how the disease manifests itself is critical to diagnosis and ultimately treatment. © 2012 Lippincott Williams & Wilkins, Inc.