The simultaneous occurrence of neuromyelitis optica spectrum disorder (NMOSD) and systemic lupus erythematosus (SLE) is an extremely rare manifestation of potentially devastating consequences, as illustrated by a recently published case report in the Journal of Medical Case Reports.

Although the probability of SLE and NMOSD coexisting in the same patient is 1 in 5 million, there are no established pathological links between the two conditions, the authors noted. NMOSD has been reported as an initial presentation of SLE in isolated case reports, they added.

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The authors reported a case involving a 24-year-old female with a history of persistent fever, polyarthralgia, skin rash, and oral ulcerations during the last two months, who was admitted due to sudden vision loss in both eyes associated with loss of muscular strength in the lower limbs. 

Physical examination was remarkable for the presence of malar rash, oral ulcers, alopecia, and swelling of the small joints of the hand. Light reflex was decreased, as well as visual acuity. Fundoscopy revealed findings suggestive of ischemic optic neuropathy. A neurological physical exam showed symmetrical weakness in both legs and sudden knee and ankle jerks with plantar extensor reflex. Sensory deficits were present up to the 8 thoracic vertebrae. 

Laboratory results revealed positive aquaporin 4 antibodies, antinuclear antibodies, and anti-ds DNA. Complement levels were decreased as expected in the context of SLE. Magnetic resonance imaging revealed findings consistent with transverse myelopathy.

The patient immediately started treatment with methylprednisolone, hydroxychloroquine, and mycophenolate mofetil. There was no significant improvement in her vision, but she regained muscle strength in the upper limbs and could walk normally. The patient is currently under follow-up care.

“Therefore, it should be distinguished first in SLE patients whether the vision problem is related to optic neuritis or ischemic optic neuropathy, is related to SLE, NMO, or MS, because treatment modalities and prognosis differ,” the authors concluded.


Sayeed, S.K.J.B., Khan, A.H., Moniruzzaman, M. et al. Systemic lupus erythematosus with acute ischemic optic neuropathy complicated with neuromyelitis optica: a case report. J Med Case Reports . Published online January 22, 2023. doi:10.1186/s13256-022-03734-8