Treatment of severe CNS lupus with intravenous immunoglobulin

We present a case of a 20-year-old woman admitted with new onset severe systemic lupus erythematosus (SLE) with multiple manifestations, including lupus nephritis with renal failure and significant central nervous system (CNS) lupus. She presented with a forbidding condition and deteriorated despite a regimen of high dose corticosteroids and cyclophosphamide administration. She had progressive CNS disease initially, with multiple generalized tonic-clonic seizures and later with dense left sided hemiparesis. After treatment with high dose intravenous immunoglobulin (IVIG), there was remarkable improvement in overall lupus disease activity and resolution of neurologic deficits. A head magnetic resonance imaging (MRI) scan early in her hospital course revealed multiple areas of vasogenic and cortical edema consistent with ischemic changes, possibly from lupus vasculitis. A repeat MRI after IVIG treatment revealed dramatic interval improvement and resolution of most of the multifocal areas of signal abnormality.The use of IVIG has been reported in a variety of autoimmune disorders, and there have also been several case reports of the successful use of IVIG in the treatment of SLE. IVIG seems to be a relatively safe treatment alternative and may have a promising role in the future of lupus treatment. We have found only five case reports in the literature specifically involving the use of IVIG in CNS lupus, but they show substantial benefits. Our case is unique because the demonstration of dramatic response to IVIG by a head MRI has not previously been reported. Based on the available information and the observations in this case, IVIG remains an experimental but promising treatment option for patients with SLE.