A Case of Coexisting Neuromyelitis Optica in Systemic Lupus Erythematosus

ABSTRACT

PURPOSE: We present a case of a patient with coexisting neuromyelitis optica and systemic lupus erythematosus (SLE). CASE SUMMARY: A 26-year-old female was hospitalized in our medical center due to decreased visual acuity in her left eye; she had a history of gastric ulcers and herpes zoster infection. Steroid treatment was started under suspicion of optic neuritis, and she was diagnosed with SLE. After treatment, her vision improved, but eleven months later she was hospitalized with paresthesia on the abdomen and left flank progressing to the lower extremities. Spinal MRI showed transverse myelitis, suggesting multiple sclerosis. Fifteen months later, the patient was hospitalized due to decreased visual acuity and ocular pain in the right eye. Her vision was improved by steroid therapy. However, optic neuritis recurred in the right eye after five weeks, thus azathioprine was added to the treatment. Anti-aquaporin-4 Ab test was conducted based on the suspicion of neuromyelitis optica, and the serum was positive for anti-aquaporin-4 Ab (NMO-IgG). The patient was hospitalized again due to paraplegia after three months. Coexistence of neuromyelitis optica was verified because spinal MRI showed longitudinally extensive transverse myelitis. The symptoms were improved by high doses of steroids, a series of plasmaphereses, and rituximab. Optic neuritis was repeated in the right eye and the symptoms were improved with high doses of steroids. Myelitis recurred later and the symptoms improved with high doses of steroids and a series of plasmaphereses. CONCLUSIONS: Coexisting neuromyelitis optica should be considered in cases with relapsing events which have transverse myelitis without cranial lesions in autoimmune diseases such as SLE.