3 DISCUSSION
The patient became severe during the process of glucocorticoid reduction, and her brain was involved in damage. Although the changes of laboratory tests were mild, the neuropsychiatric symptoms and brain MRI were progressive. The symptoms became worse, even if intensive immunosuppressive and biological agents were prescribed. It was worthy of note that, the brain MRI was rapidly improved after two sessions of IAS were performed, together with intravenous cyclophosphamide and immunosuppressive therapy.
Glucocorticoid combination with intravenous pulse cyclophosphamide is the most classical strategy for SLE with major organs involved [5]. It’s effective in many case, but not all situations. When common strategies are contraindicated or fail in life-threatened SLE, extracorporeal treatments are performed. Although plasma exchange could non-selectively remove the antibodies, it is reported to be not effective in prospective trail [6]. In contrast, IAS can reduce significantly the concentration of IgG in blood without loss of coagulation factors and plasma albumin. Stummvoll and colleague found that IAS improved significantly disease activity and lowered glucocorticoid dosages in SLE patients [3,7]. These patients showed benefits within 3 months of treatment and stabilization thereafter. IAS might be used as an optional treatment when other therapies are not effective in SLE [4].
NPSLE is associated with a worse prognosis, and brain MRI scan is recommended to evaluate brain lesions in patients [8]. The most frequent MRI findings are focal lesions in subcortical white matter, cortical atrophy, diffuse cortical grey matter changes, less brain and corpus callosum volumes in patients with NPSLE [9,10]. The patient showed abnormal hyper-intense in right basal ganglia at the beginning, similar to cerebral infarction. When the disease progressed, atrophy of the brain, diffuse white matter and multiple nuclear lesions were identified. The formation of these manifestations contribute to blood-brain barrier dysfunction, cerebrovascular disease, serum and CSF autoantibody-mediated injury [11]. Microinfarcts may be responsible to retinal vasculitis and basal ganglia lesions in this patient. The auto-antibodies deposition in the brain play an important role in the pathogenesis [12]. Some of them, such as antiphospholipid antibodies, are reported more likely to be elevated in NPSLE [13]. Besides, evidences suggest that these immunoglobulins and immune cells could leak into brain tissue and cause immune responses.
The ability of elimination of circulating complexes by protein A IAS was confirmed in several autoimmune diseases [14-16]. Braun and colleague reported the elimination kinetics of IgGs and circulating immune complexes in vivo during the treatment of severe SLE [17]. It showed that all IgG subclasses were removed from the patient’s plasma. The half time of IgG elimination is 4.8 days during intermittent therapy. Protein A IAS decreases IgG levels in the blood, leads to a reduction IgG in cerebrospinal fluid, and then improves the clinical symptoms of NPSLE. The immunosuppressive treatment could reduce the recomposition and redistribution. Thus the treatment of adsorption could be better, when immunosuppressive agents are prescribed. Because of competition for protein A binding sites, intravenous immunoglobulin prevents the rapid decline of IgG in the patients,which is suggested to be avoided during protein A IAS treatment[17].