Introduction: Neuropsychiatric systemic lupus erythematosus (NPSLE) is characterized by a heterogeneity of clinical manifestations. The absence of diagnostic criteria and the lack of clinical trials is a challenge in clinical practice.
Areas covered: A literature review was performed to describe epidemiology, characterization (clinical, immunological, and imaging), diagnosis and treatment of NPSLE. Classification criteria have been the first step towards a uniform definition. More recently, different attribution models have been developed to help to determine if the NP event is due to SLE. Disease activity is a major risk factor for NP events. Cytokines and autoantibodies are associated with NP events, however, only a few studies have identified risk factors for individual NP events.
Expert opinion: Further research needs to search for and validate biomarkers for NPSLE and individual NP events, including neuroimaging findings, attribution models, and serologic markers. This will be a fundamental step in planning randomized control trials in the treatment of NPSLE to improve outcome. 相似文献
Summary A 29 year-old woman with SLE was admitted to our department due to severe remitting headaches. Following investigation a high degree of intra cranial pressure was determined.Several years ago a similar finding was diagnosed, and the disease was brought into remission by the administration of periodical pulses of high dose intravenous immunoglobulins.Benign intracranial hypertension (BIH) is an uncommon presentation of neuropsychiatric SLE. In this patient several risk factors of BIH (obesity, steroid therapy, and SLE) assembled and elicited a severe presentation of the disorder which became more resistant to therapy. Several pathogenic pathways tie BIH with SLE as thrombotic obliteration of cerebral arteriolar and venous systems and immune complex deposition within the arachnoid villi (that are responsible for CSF absorption). As shown in this case report of BIH, clinical findings do not always parallel various imaging techniques as MRI and CT brain scans. 相似文献