IVIg in Cerebral Lupus

How often is one left with a dilemma when a patient is too sick to receive further immunosuppression but requires intervention for an active lupus activity? This is compounded by the paucity of data especially in the treatment of cerebral lupus. There is no large randomised trial to guide treatment and much depends on the experiences from case reports.


Here we look at the evidence of IVIg in the treatment of cerebral lupus. We will be revisiting this article as we go along,


Clin. Rheumatol. 1999; vol. 18(2) pp. 170-3

Successful treatment of systemic lupus erythematosus cerebritis with intravenous immunoglobulin.

Sherer Y, Levy Y, Langevitz P, Lorber M, Fabrizzi F, Shoenfeld Y

Neuropsychiatric lupus includes extremely diverse clinical manifestations, ranging from mild cognitive dysfunction to a severe, life-threatening presentation. We report a 28-year-old patient with systemic lupus erythematosus who had persistent fever for 3 months, and developed within a few hours motor and sensory aphasia, rotator nystagmus with deviation of the eyes, and severe nuchal rigidity. An extensive series of imaging and laboratory tests were interpreted as normal, except for an elevated opening pressure at lumbar puncture, cerebrospinal fluid inflammatory findings, and asymmetrical cortical perfusion on single-photon emission computed tomography. The patient received one course of high-dose intravenous immunoglobulin (IVIg) and within 5 days her condition returned to that of 3 months before admission. The mechanisms of injury, along with the management of cerebral lupus and the mechanisms of action of IVIg, are discussed.

PMID: 10357127
URL  – http://www.ncbi.nlm.nih.gov/pubmed/10357127?dopt=Citation


J Clin Rheumatol. 2009 Jun; vol. 15(4) pp. 181-4

Transient life-threatening cerebral edema in a patient with systemic lupus erythematosus.

Bianchi MT, Lavigne C, Sorond F, Bermas B

Central nervous system symptoms occur in a substantial portion of patients with systemic lupus erythematosus. However, coma is a rare presentation and is usually secondary to complications such as subarachnoid hemorrhage, seizure, or ischemia. Here, we present a 49-year-old woman with lupus erythematosus and a history of recurrent aseptic meningitis and mild subarachnoid hemorrhage who presented with altered mental status and lethargy that progressed rapidly over hours to the herniation syndrome of coma, extensor posturing, and unilateral pupillary dilation. Spinal fluid showed massive protein elevation (>1600), and head computed tomography revealed global cerebral edema. The clinical and radiologic findings rapidly reversed with intravenous corticosteroids and mannitol within 24 hours, and her mental status improved to baseline. Her course was complicated by 2 episodes of recurrent encephalopathy when corticosteroids were tapered; these resolved after resuming high dosing. Because of ongoing pancytopenia, chemotherapy immunosuppression was delayed, and instead she received intravenous immunoglobulin with improvement in the pancytopenia. She remained cognitively intact during subsequent corticosteroid tapering. Rapid development of coma in lupus patients may be due to a primary process of the disease impacting blood brain barrier integrity. Although rare, this potentially fatal complication may be reversible with acute corticosteroid administration.

PMID: 19455059
URL  – http://www.ncbi.nlm.nih.gov/pubmed/19455059?dopt=Citation