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. 2015:2015:324365.
doi: 10.1155/2015/324365. Epub 2015 Mar 9.

Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

Teresa Carbone  1 , Ricardo Azêdo Montes  2 , Beatriz Andrade  1 , Pedro Lanzieri  1 , Luis Mocarzel  1
Affiliations

Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

Teresa Carbone et al. Case Rep Rheumatol. 2015.

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.

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Figures

Figure 1
Figure 1
CT scan showing diffuse infiltrate of orbital tissue and muscle inflammation. Compare stages before (a) and after (b) immunosuppressive treatment.
Figure 2
Figure 2
Histological sample with hematoxylin and eosin stain showing irregularly whorled pattern of fibrosis (storiform fibrosis) and lymphoplasmacytic infiltrate in the interstitium.
Figure 3
Figure 3
Before (a) and after (b) treatment with glucocorticoids.
See this image and copyright information in PMC

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