Table of Contents Author Guidelines Submit a Manuscript
Case Reports in Rheumatology
Volume 2015, Article ID 324365, 4 pages
http://dx.doi.org/10.1155/2015/324365
Case Report

Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

1Department of Internal Medicine, Hospital Universitário Antônio Pedro (HUAP), Universidade Federal Fluminense (UFF), Rua Marquês de Paraná 303, 7° andar, Centro, 24033-900 Niterói, RJ, Brazil
2Department of Rheumatology, Hospital Universitário Antônio Pedro (HUAP), Universidade Federal Fluminense (UFF), Rua Marquês de Paraná 303, 7° andar, Centro, 24033-900 Niterói, RJ, Brazil

Received 9 January 2015; Revised 23 February 2015; Accepted 26 February 2015

Academic Editor: Suleyman Serdar Koca

Copyright © 2015 Teresa Carbone et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.