Systemic manifestations and pathogenesis exploration of neuromyelitis optica spectrum disorders

Objective To investigate the immunological abnormalities beyond central nervous system (CNS) associated with neuromyelitis optica spectrum disorders (NMOSDs). Methods Clinical data of 56 patients with NMOSDs from January 2010 to December 2013 enrolled in Department of Neurology at Peking University People's Hospital were analyzed retrospectively. All patients were divided into 2 groups: neuromyelitis optica (N = 33) and non-neuromyelitis optica (N = 23). Each patient underwent detailed physical examination including internal medicine and nervous system. Records of complicated autoimmune diseases as well as scoring of Expanded Disability Status Scale (EDSS) were used to evaluate disease severity. Part of patients received detection of multiple immunological indicators. Results In all patients with NMOSDs, there were 3 cases with Hashimoto thyroiditis, one case with systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS), asthma, hyperthyroidism, rheumatoid arthritis and iridocyclitis, respectively. In patients whose immunological indices were available, the first three abnormal immunological changes were abnormal thyroid function (10/17), positive anti-nuclear antibody (14/28) and positive complement 3 (8/19). In addition, NMOSDs got worsen in 2 cases after delivery. Conclusions NMOSDs coexist with many kinds of autoimmune diseases and multiple autoantibodies. The above-mentioned autoimmunity may be related to the pathogenesis of NMOSDs. Besides, pregnancy or delivery may aggravate the disease severity of NMOSDs. doi: 10.3969/j.issn.1672-6731.2014.09.009