Systemic lupus erythematosus with neuromyelitis optica：a case report and literature review

Abstract:

Objective：To analyze the clinical characteristics，treatment and prognosis of systemic lupus erythematosus（SLE） with neu－romyelitis optica（NMO）. Methods：Clinical manifestation，laboratory examination，and diagnosis and treatment of one child with SLE and NMO in our hospital was summarized，and clinical data of 30 reported cases about SLE with NMO at home and abroad were analyzed in combination with our case. Results：①The patient was 10 years and 8 months old，with onset of skin rash and arthritis. He developed progressive muscle weakness，impaired vision，proteinuria，positive serum autoantibody to nuclear antigen（ANA），positive anti-NMO-immunoglobuin G（IgG） antibody and abnormal edema pattern in the whole spinal cord，with final diagnosis of SLE with NMO. He was treated by methylprednisolone pulse and immuneglobulin（IVIG） pulse，oral mycophenolate mofetil（MMF），and intra－venous rituximab（RTX）. After treatment，his vision and myodynamia were gradually recovered，without relapse after six months of fol－low-up. ②Combing with literatures，the most common clinical manifestations among those 31 patients were impaired vision（27/31），muscle weakness（24/31） and paresthesia（21/31）. All patients had positive ANA and abnormal MRI signals of spinal cord，including positive double-stranded DNA（dsDNA） antibody（17/26），hypocomplementemia（13/24） and positive NMO-IgG antibody（24/26）. ③All patients were treated with glucocorticoid；treatment in the acute phase included methylprednisolone pulse（27/31），lasmaphere－sis （11/31），IVIG（9/31） and so on. All 30 patients were treated with immunosuppressants，including cyclophosphamide（CTX） pulse （15/31），intravenous RTX（10/31），azathioprine（AZA）（8/31），and oral MMF（7/31）. ④Those 12 patients treated with CTX had a following up for more than half a year，including relapse of 6 patients（31 times of relapse，including 31 times of myelitis and 2 times of optic neuritis）；among 10 patients treated with RTX，8 patients had no recurrence and 4 patients had a re－duced annual recurrence rate. Conclusion：SLE complicated with NMO is characterized by repeated polyphase course with high re－currence rate，is also prone to complications such as paralysis and blindness and etc. and positive rate of serum NMO-IgG antibody and spinal MRI is high. Early methylprednisolone pulse in combination with RTX can reduce the frequency of recurrence，and signifi－cantly improve the condition and prognosis.