Perivascular fibrosis and IgG4-related disease: a case report

  • S. Monti | saram86@libero.it U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
  • G. Crepaldi U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
  • A. Peri U.O. Chirurgia Generale, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
  • A. Pietrabissa U.O. Chirurgia Generale, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
  • P. Morbini U.O. Anatomia Patologica, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
  • F. Bobbio-Pallavicini U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
  • C. Montecucco U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
  • R. Caporali U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.

Abstract

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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Published
2014-11-06
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Issue
Section
Case Reports
Keywords:
Immunoglobulin G4-related disease, Perivascular fibrosis, Retroperitoneal fibrosis, Aortic aneurysm.
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How to Cite
Monti, S., Crepaldi, G., Peri, A., Pietrabissa, A., Morbini, P., Bobbio-Pallavicini, F., Montecucco, C., & 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