Challenging differential diagnosis, Management of emergency care, Rare disease
Alexander J. Heckman, Ali A. Alsaad, Michael W. Stewart, Michael J. Maniaci
Department of Internal Medicine, Mayo Clinic in Florida, Jacksonville, FL, USA
Am J Case Rep 2019; 20:97-100
Systemic lupus erythematosus (SLE) causes sight-threatening, ophthalmologic problems that are frequently challenging to manage. Optic neuropathy is a rare ophthalmological complication of SLE that can progress to total bilateral vision loss if not identified and treated rapidly. We describe a patient with SLE who presented with an acute, painless unilateral optic neuropathy who subsequently experienced partial recovery of vision when treated with high-dose intravenous corticosteroids.
CASE REPORT: A 44-year-old female with known SLE presented with 4 days of painless, complete, and gradual vision loss in the right eye. Initial ophthalmologic examination revealed no light perception, afferent pupil defect, 4+ optic disc swelling, and 1+ venous tortuosity of the right eye. No hemorrhage or exudates were noted. Diagnostic workup revealed a lupus flare with elevated inflammatory markers including elevated anti-nuclear antibody, anti-ds-DNA antibody, anti-Sm antibody, and anti-phospholipid IgG antibody. The diagnosis of optic neuropathy was clinically established, and the patient was treated with high-dose intravenous corticosteroids. Her vision improved, and she was transitioned to oral corticosteroids with eventual significant improvement in her vision.
CONCLUSIONS: Optic neuropathy is a rare and devastating ophthalmologic complication of SLE. The diagnosis can be made by linking key clinical findings on ophthalmologic examination with positive serological studies. If treated rapidly with immunosuppressive therapy, the vision loss can be reversed, and permanent blindness avoided. Although this rare complication is generally bilateral in nature, clinician must also be aware of unilateral disease and treat patients accordingly.
Keywords: Deaf-Blind Disorders, Lupus Erythematosus, Systemic, Optic Nerve Diseases, Optic Neuropathy, Ischemic