Optic Neuritis as the First Manifestation of Rheumatoid Arthritis

Optic neuritis has been rarely reported in patients with rheumatoid arthritis (RA) and never as the initial manifestation in a patient who lacked any clinical evidence of RA until years later. We report a patient with optic neuritis as the initial presentation of RA. A 52-year-old woman without medical problems presented in 1988 to our hospital with sudden left eye visual loss for 3 weeks. Best-corrected visual acuity was 20/25 in the right eye and no light perception in the left eye. Optic disc swelling was present in the left eye. The right eye visual field was normal. Laboratory studies showed a normal erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level. Normal results included tests for antinuclear antibodies (ANAs), rheumatoid factor (RF), C3, and C4. Orbit CT revealed no abnormalities. Despite oral corticosteroid treatment for presumed optic neuritis, visual acuity of the left eye did not recover. In 1995 she complained of poor vision of the right eye for 3 days. Best-corrected visual acuity was 20/200 in the right eye and no light perception in the left eye. The right optic disc was normal, and the left optic disc was pale. She read none of the Ishihara color plate. Automated perimetric examination revealed a nasal upper quadrant defect. Brain CT was normal (MRI was contraindicated because of residual bomb chips in her abdomen and mediastinum). ESR and CRP level were normal, the RF level was elevated and results for ANA were positive, with normal levels of C3 and C4. Pulse therapy with methylprednisolone produced recovery to 20/20 and a normal visual field in the right eye. In 1999, she developed arthralgia and symmetrical swelling with morning stiffness over the proximal interphalangeal joints and carpal bones of both hands. Plain x-rays of the both hands showed periarticular swelling in the proximal interphalangeal joints and relatively osteoporotic changes of carpal bones. She had an elevated RF level and a positive results for ANAs. RA was diagnosed according to the clinical classification criteria for RA (1). She began treatment for this condition. In 2006 she reported acute blurred vision of the right eye again for 3 days but no exacerbation of rheumatologic symptoms. Best-corrected visual acuity of the right eye was finger counting. No optic swelling of the right eye was found. Perimetry of the right eye revealed nerve fiber bundle defects. RF level and test results for ANAs were normal, but her test results were positive for anti-Ro and anti-La antibodies. Pulse therapy with methylprednisolone produced recovery of visual acuity in the right eye to 20/20 but both optic discs were now pale (Fig. 1) and visual field loss persisted.FIG. 1: After bouts of optic neuropathy in both eyes, the optic discs are pale bilaterally.Optic neuropathy was first reported in RA in 1980 (2). Autopsy showed necrotizing vasculitis of the right posterior ciliary artery and lymphocytic vasculitis and perivasculitis of the left posterior ciliary artery (2). Approximately 25% of patients with RA have vasculitis with involvement in all sizes of veins and arteries on post-mortem examination (2). Occlusion of one of the posterior ciliary arteries or its branches produces ischemic optic neuropathy with a spectrum of changes that results in complete blindness or variable visual field defects (2). Milder optic nerve damage due to a vasculitic mechanism, characterized by demyelination, had a good response to pulse corticosteroid therapy (3). More severe and irreversible cases of optic neuropathy have caused axonal necrosis (3). In our patient, the first attack of optic neuropathy of the left eye resulted in blindness. Its features are atypical for optic neuritis and more typical of a severe ischemic process. In subsequent attacks affecting the right eye, however, vision did recover and corticosteroid treatment might have made a difference, perhaps by attenuating vasculitis. Particularly unusual in our patient is the fact that the manifestations of RA appeared 11 years after the first attack of optic neuropathy. We highlight this patient to emphasize that an underlying rheumatologic condition may be occult and that early treatment with corticosteroids of a presumed vasculitis may be vision-saving. Ying-Hua Chen, MD An-Guor Wang, MD Yen-Ching Lin, MD May-Yung Yen, MD Taipei Veterans General Hospital Taipei, Taiwan [email protected]