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Autoimmun Rev. 2014 Dec;13(12):1203-10. doi: 10.1016/j.autrev.2014.08.013. Epub 2014 Aug 22.

The clinical spectrum of IgG4-related disease.

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Josep Font Laboratory of Autoimmune Diseases, CELLEX-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Spain.
Department of Internal Medicine (ICMiD), Hospital Clínic, Barcelona, Spain.
Harvard Medical School and Department of Medicine, Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Boston, MA 02114, USA. Electronic address:


IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease with the capability of involving essentially any organ. The epidemiology of this disease has not been explored in detail. A majority of patients reported in the literature to date are from Japan, but the condition has been described all across the world and there is no strong evidence to suggest a predilection for Asian populations. The mean age at diagnosis is approximately 60 years and there is a decided male predominance for many clinical features, with an overall male:female ratio of 8:3. A cardinal feature of IgG4-RD is single or multiple organ swelling that often raises concern for malignancy. IgG4-RD should be suspected in patients presenting with unexplained enlargement or swelling of one or more organs. Presenting features vary substantially according to the specialty to which patients present first; in addition, the disease can be diagnosed unexpectedly in pathological specimens or identified incidentally on radiology studies. Involvement of major organs is common and IgG4-RD may lead to organ failure, particularly in the pancreas, liver and biliary tree, kidneys, thyroid gland, lungs, and aorta. The diagnosis of IgG4-RD relies on the coexistence of various clinical, laboratory and histopathological findings, although none is pathognomonic by itself.


Aortitis; Autoimmune pancreatitis; IgG4-related disease; Mikulicz disease; Retroperitoneal fibrosis; Sclerosing cholangitis

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