Steroid-Responsive Optic Neuropathy (SRON) in Anti-neutrophil Cytoplasmic Antibody–Negative Wegener Granulomatosis with False-Positive Indicators of Giant Cell Arteritis and Sarcoidosis

Abstract

A 65-year-old woman with rheumatological symptoms and diplopia was diagnosed as suffering from giant cell arteritis. Four years later she presented to us with a steroid responsive optic neuropathy. A presumptive diagnosis of neurosarcoidosis was made because of a gallium-67 single-photon emission tomography scan indicating a positive ‘panda sign’. Subsequent chest computerised tomographic imaging revealed cavitating lung lesions and an open biopsy established the diagnosis of Wegener granulomatosis despite the negative anti-neutrophilic cytoplasmic antibody. At the time of diagnosis the patient had a steroid responsive optic neuropathy and had recently developed a bilateral uveitis. Treatment with cyclophosphamide and corticosteroids produced complete remission with no further progression of disease.

KEYWORDS::

• optic neuropathy
• steroids
• Wegener granulomatosis

ACKNOWLEDGEMENT

Research to Prevent Blindness (RPB) provided unrestricted research support.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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