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Systemic Inflammatory Pseudotumor and Myasthenia Gravis

Marc H. Levin, MD, PhD1,2; Pallavi P. Gopal, MD, PhD3; Steven L. Galetta, MD4
[+] Author Affiliations
1Scheie Eye Institute, Department of Neurology and Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia
2now with Department of Ophthalmology, University of California, San Francisco
3Department of Pathology, Perelman School of Medicine, University of Pennsylvania, Philadelphia
4Department of Neurology, NYU Langone Medical Center, New York University, New York
JAMA Ophthalmol. 2014;132(3):359-361. doi:10.1001/jamaophthalmol.2014.16.
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Extract

We describe a patient with aggressive systemic inflammatory pseudotumor manifesting as isolated ocular myasthenia gravis. In this case, the routine chest imaging performed following the diagnosis of myasthenia gravis identified occult mediastinal lymphoid disease years prior to development of diffuse systemic symptoms.

Article InformationCorresponding Author: Marc H. Levin, MD, PhD, Department of Ophthalmology, University of California, San Francisco, 10 Koret Way, K203, San Francisco, CA 94143 (levinm@vision.ucsf.edu).

Author Contributions: Dr Galetta had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Study concept and design: Levin, Galetta.

Acquisition of data: All authors.

Analysis and interpretation of data: All authors.

Drafting of the manuscript: Levin.

Critical revision of the manuscript for important intellectual content: All authors.

Administrative, technical, and material support: Levin, Gopal.

Study supervision: Galetta.

Conflict of Interest Disclosures: Dr Galetta has received consulting honoraria from Biogen Idec and Teva Pharmaceuticals. No other disclosures were reported.

Funding/Support: Dr Levin was supported by the Heed Ophthalmic Foundation.

Role of the Sponsor: The funding organization had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Previous Presentation: This paper was presented at the 39th Annual Meeting of the North American Neuro-Ophthalmology Society; February 10, 2013; Snowbird, Utah.

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Figure 1.
Lung and Kidney Lesions Concerning for Metastatic Renal Cell Carcinoma

A, Axial chest computed tomography incidentally showed dense right lower lobe consolidation and multiple patchy confluent nodules present in both lower lobes. B, Coronal abdominal magnetic resonance imaging demonstrates a 6 × 8 × 10-cm left kidney mass (arrowheads) surrounding much of the collecting system and proximal ureter and extending into the pararenal space.

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Figure 2.
Kidney and Lung Pathology Consistent With Systemic Inflammatory Pseudotumor

A, Left nephrectomy specimen revealed a kidney at end stage with globally sclerosed glomeruli with fibrotic areas and lymphoplasmacytic infiltrates (hematoxylin-eosin, original magnification ×10). B, The kidney showed scattered IgG4-positive cells (original magnification ×20). C, Excisional wedge biopsy of a left upper lung nodule showed an atypical lymphoid infiltrate with heterogeneous inflammatory cells within a fibrotic background (hematoxylin-eosin, original magnification ×10).

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