Perivascular fibrosis and IgG4-related disease: a case report

Submitted: 23 May 2014
Accepted: 30 August 2014
Published: 6 November 2014
Abstract Views: 1853
PDF: 1012
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Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibroinflammatory condition which can potentially involve any organ. Some characteristic histopathologic features with lymphoplasmacytic infiltrate, an increased number of IgG4+ cells, storiform fibrosis and obliterative phlebitis are the mainstay for diagnosis. Serum IgG4 levels often increase. We report the case of a patient with perivascular fibrotic lesions involving the aortic arch and the splenic hilum, with a surgical biopsy-proven diagnosis of IgG4-related disease. The patient is now undergoing a low-dose corticosteroid maintenance therapy without evidence of new localizations of the disease. This case highlights the need for increasing awareness and recognition of this new, emerging clinical condition.

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How to Cite

Monti, S., Crepaldi, G., Peri, A., Pietrabissa, A., Morbini, P., Bobbio-Pallavicini, F., … Caporali, R. (2014). Perivascular fibrosis and IgG4-related disease: a case report. Reumatismo, 66(3), 245–248. https://doi.org/10.4081/reumatismo.2014.753

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