Adjunctive plasma exchanges to treat neuropsychiatric lupus: a retrospective study on 10 patients

Abstract

Neuropsychiatric manifestations of systemic lupus erythematosus (NPSLE) are among the main causes of morbidity and mortality attributed to lupus activity. Conventional NPSLE treatment combines CS and immunosuppressants, but some symptoms do not respond. We retrospectively evaluated the adjunction of plasma exchanges (PE) to treat 13 NPSLE flares occurring in 10 patients (mean age, 30 years) between 1989 and 2002. NP manifestations were the first SLE symptoms for seven patients, with a mean of 3.2 NP manifestations/flare. All patients received CS and cyclophosphamide pulses. A mean of 15 PE/flare were performed. All patients improved within a mean of 3 (median: 2.5; range: 1.5-8) weeks thereafter. Complete remissions of 7/13 flares were obtained within a mean of 7 (median: 4; range: 2-22) weeks. Partial remissions were achieved for the remaining six flares, characterized by new NP manifestations during three and insufficient control of the others. Other SLE manifestations regressed for all patients with the mean European consensus lupus activity measurement score declining from pretreatment 6.9 to 1.2. A regimen combining CS, cyclophosphamide and PE is effective against severe NPSLE, with acceptable toxicity.

EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs

“…When NPSLE is thought to reflect an inflammatory/neurotoxic process (especially aseptic meningitis, optic neuritis, transverse myelitis, peripheral neuropathy, refractory seizures, psychosis, acute confusional state; ACS) and in the presence of generalised lupus activity, management includes glucocorticoids alone or in combination with immunosuppressants (azathioprine or cyclophosphamide) 24 25. In severe NPSLE refractory to standard immunosuppressive therapy, among other treatments, plasma exchange,26 27 intravenous immunoglobulin,28–30 and rituximab (anti-CD20 monoclonal antibody)31 have been used.…”

“…A combination of glucocorticoids with immunosuppressive agents is effective in most patients (response rates up to 70%) 25 63 64. Plasma exchange therapy (synchronised with intravenous cyclophosphamide)26 27 and rituximab have been used in refractory cases.…”

“…Relapses are common (50–60%) during corticosteroid dose reduction, underscoring the need for maintenance immunosuppressive therapy. Plasma exchange therapy has been used in severe cases26 27 76 and anticoagulation therapy in antiphospholipid-positive myelopathy with good results 37 38. Factors associated with severe neurological deficit include extensive spinal cord MRI lesions, reduced muscle strength or sphincter dysfunction at presentation, antiphospholipid antibodies, and delay (>2 weeks) in the initiation of therapy 76 77…”

Acute inflammatory myelopathies

“…EULAR recommendations also consider plasmapheresis for cases refractory to induction therapy (Neuwelt, 2003;Bartolucci et al, 2007). In addition, plasmapheresis may be beneficial in patients in whom corticosteroids are contraindicated (Yucesan et al, 2007).…”

Approach to Patients with SLE Presenting with Neurological Findings

“…Those include plasma exchange, rituximab or intravenous immunoglobulin. The combination of the treatment modalities ( cyclophosmaide/corticosteroid/ plasma exchange (Bartolucci et al,2007) or plasmapheresis alone or with cyclophosphamide (Neuwelt,2003)) was evaluated in small series of refractory disease. Results from those showed favourable outcome with combination modalities.…”