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IgG4-Associated Vasculitis

  • VASCULITIS (LR ESPINOZA, SECTION EDITOR)
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Abstract

Elevated IgG4 is characteristic of cases of IgG4-RD, a newly recognized systemic disease. However, several chronic inflammatory conditions, including rheumatic diseases, can also be associated with increased levels of IgG4. There have also recently been several reports describing an increased IgG4 immune response to some vasculitis syndromes, in particular Churg–Strauss syndrome and granulomatosis with polyangiitis. To avoid misdiagnosis, clinicians must be aware that the clinical manifestations of IgG4-RD and ANCA-associated vasculitis may overlap. The meaning of these observations is not yet understood, and more studies are needed to determine the true significance of the increased IgG4 response to vasculitis syndromes, especially anti-neutrophil cytoplasmatic antibodies (ANCA)-associated vasculitis.

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Rodolfo Perez Alamino declares that he has no conflict of interest.

Carlos Martínez declares that he has no conflict of interest.

Luis R. Espinoza declares that he has no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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Correspondence to Luis R. Espinoza.

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This article is part of the Topical Collection on Vasculitis

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Perez Alamino, R., Martínez, C. & Espinoza, L.R. IgG4-Associated Vasculitis. Curr Rheumatol Rep 15, 348 (2013). https://doi.org/10.1007/s11926-013-0348-9

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  • DOI: https://doi.org/10.1007/s11926-013-0348-9

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