Objective To investigate the clinical features of neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSD) with primary Sj(o)gren' s syndrome (pSS).Methods Eleven inpatients diagnosed as NMO secondary to pSS in Peking Union Medical College Hospital were retrospectively analyzed.Results Eleven patients of pSS with NMO were enrolled in this study,including 10 females and 1 male.The mean age was(35 ± 15)years old.The course of disease ranged from one month to 16 years,with a median of 10 months.The interval between optic nerve and spinal cord involvement was 3 months to 2 years.Eight patients had NMOSD as initial presentations of pSS.Eight patients had history of optic neuropathy.The most frequently involved spinal segment was cervical cord (10 cases) shown by magnetic resonance.Serum NMO-IgG antibodies were tested in 7 patients and 6 of them were positive.Conclusion NMOSD may present as an important and initial clinical manifestation of pSS,which suggesting the involvement of central nervous system.Autoimmune antibodies,NMO-IgG and imaging were supposed to be done for further evaluation of prognosis and therapy regimens.