Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease.

Case Rep Rheumatol

Department 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.

Published: April 2015

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.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4369927PMC
http://dx.doi.org/10.1155/2015/324365DOI Listing

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