Central nervous system involvement in primary Sj(o)gren's syndrome: a study of 38 patients / 中华风湿病学杂志

ABSTRACT

Objective To analyze the clinical profile of primary Sj(o)gren's syndrome (pSS) patientswith central nervous system (CNS) involvement.Methods Thirty-eight pSS-CNS patients at Nanjing Drum Tower Hospital between 2012 and 2016 were enrolled.These patients were divided into high activity group and moderate activity group,according to European League against Rheumatism Sj(o)gren's syndrome Disease Activity Index (ESSDAI).The imaging characteristics,clinical features,laboratory examinations and treatment of 38 cases were retrospectively analyzed.Quantitative differences were analyzed by the student's t-test and qualitative data were analyzed with chi-square or Fisher's exact test.Results The prevalence of central nervous system involvement in pSS patients was 2.93％(38/1 296),while 32％(12/38) as the initial symptom of pSS.Recurrence rate was 39％(15/38).Limb weakness,speech difficulty,sensory disorders,blurred visionwere the most frequent symptoms in pSS-CNS.Multiple lesions in Magnetic resonance imaging (MRI) examination and cerebrospinal fluid abnormality were seen in 94％ (15/16) pSS-CNS patients,among which intra-thecal IgG level increased in 50％ (8/16).In addition,the frequencies of lung involvement,immune associated thrombocytopenia,high-titer antinuclear antibody (ANA) were significantly higher in high activity group of pSS-CNS than those of moderate activity group.The value of above items was 4.7,5.0 and 5.3,respectively,and all the differences were significant (P＜0.05).After high-dose corticosteroids and immunosuppressive therapy,61％ (23/38) patient improved,37％(14/38) were unresponsive to treatment,and 3％(1/38) died because of acute massive cerebral infarction.For those unresponsive patients,mesenchymal stem cell transplantation or immune adsorption treatment might be effective.Conclusion The clinical manifestations of central nervous system are diverse,may be the initial presentations in some pSS patients,with low morbidity and high recurrence rate.Image and lumbar puncture are important for diagnosis.Pulmonary involvement,immune thrombocyto-penia,and high-titer ANA are frequently associated with the activity of pSS-CNS.Most patients have good response to the treatment regimens of high-dose corticosteroids and immunosuppressive therapy,mesenchymal stem cell transplantation or immuno-sorption therapy may be considered in those unresponsive cases.