Objectives To assess the effect of Rituximab (RTM) in patients with neuropsychiatric SLE (NPSLE) refractory to conventional treatment during long-term follow-up.
Methods Various clinical manifestations of central and peripheral nervous system (CNS, PNS) involvement were observed in 22 patients, 18 of them had CNS involvement (cranial neuropathies in 8 patients, cognitive dysfunction-7, transverse myelitis–6, lupus headache–5, seizure disorders–4, optic neuritis symptoms-4, petit mal seizures-2, psychosis–2, cerebrovascular accident-1). Some of them had combined symptoms of CNS involvement. All of 22 patients had the widest spectrum of PNS involvement, which were confirmed by electroneuromyography.
SLE activity was assessed by SLEDAI2K. The median of SLEDAI2K before the treatment was 22 [12-27]. Extremely severe and prognostically unfavorable course of the disease has been observed in 6 patients with NPSLE and active lupus nephritis. Previous therapy with cyclophosphamide (CF) received 14 patients with CNS involvement with a total dosage of 5,0 [1,0-10,0]g. The remaining 4 patients received monotherapy with glucocorticoids (GC). 4 patients also received azathioprine (AZA), 5 - hydroxychloroquine, 3 - mycofemolate mofetil (MFM), 7–high dosage of glucocorticoids (GC) (above 40mg/day). The first course of RTM was performed by this scheme: 8 patients received 1000mg RTM, 12 patients 2000 mg, 2 patients 1500 mg. 6 of them received puls-therapy with GC and CF with first course of RTM. After the first course of RTM the half of the patients received maintenance therapy with cytostatics: CF-5, AZA-3 and MFM-3 patients. Single course of RTM received 9 patients, the remaining 13 patients received repeated courses of RTM during the long-term of follow-up.
Results 19 (86%) patients achieved a clinical response (13 complete response, 6 partial response) during the 15 [12-42]months of follow-up. 2 patients didn't respond to the therapy, 1 died due to infection during a month after therapy. The exacerbation of the CNS symptoms was occurred in 2 patients (9%) (at 48 and 60 months respectively) during all the period of the follow-up. The flares were treated with repeated courses of RTM.
The decreasing the activity of lupus was associated with the reduction of the number of B-cells, SLEDAI2K, a-dsDNA and steroid-sparing effect. All patients had depleted peripheral B-cells after a month of therapy with RTM. The average time of the recovery of B-cells after the first course of RTM was 6 [4.5-12] months.
Conclusions Anti-B-cell therapy with Rituximab improved various clinical, laboratory signs and symptoms in 86% of patients with NPSLE refractory to conventional treatment during long-term follow-up. Rapid response was observed in 83% of patients with CNS lesions during the first month after the therapy. Reduction of PNS symptoms was observed immediately and was continued during 6 months after the first course of rituximab. A significant steroid-sparing effect was noticed.
Disclosure of Interest None declared