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].