Background Neuropsychiatric involvement of systemic lupus erythematosus (NPSLE) is one of the most deleterious complications of the disease, leading to great decrease of quality of life and poor prognosis . Factors such as activity of disease have been associated NPSLE [1,2]. The current treatment of NPSLE remains mostly empiric, requiring high-dose corticosteroids and extensive immunosuppressive therapy [3,4]. Short-term efficacy of treatments in NPSLE have been evaluated by several studies, however few studies have been performed to evaluate long-term efficacy of these drugs .
Objectives To compare the long-term effectiveness among drugs and associated factors in the treatment of NPSLE.
Methods 209 patients (≥14 years) from a Mexican cohort from 2011 to 2017 were examined. SLE and NPSLE cases fulfilled 1997 ACR and 1999 ACR criteria respectively. Demographic factors, comorbidities and pharmacologic treatments were reviewed for patients with NPSLE. Variables were studied by bivariate, multivariate and survival analyses.
Results Of 209 SLE patients, 37 (17.7%) had NPSLE; of them 32 were women. The mean age [standard deviation (SD)] was 38.8 (14.6) years. The mean of time at onset of SLE (SD) was 6.2 (5.4) years. SLEDAI and SLICC mean (SD) were 22.1 (10.2) and 1.83 (1.2) separately. Diffuse and focal manifestations of NPSLE were presented in 20 and 17 cases. Central and peripheral nervous system events of NPSLE were described in 34 and 3 of patients. The NPSLE manifestations more prevalent were cerebrovascular disease (n=15), psychosis (n=8) and seizure disorders (n=5). A total of 112 cycles of treatment were analyzed [prednisone (PDN) n=30, intravenous (iv) methylprednisolone (MTP) n=23, iv cyclophosphamide (CYC) n=13, azathioprine (AZA) n=12, mycophenolate mofetil (MMF) n=11, rituximab (RTX) n=10, hydroxichloroquine (HCQ) n=9, plasmapheresis (PPH) n=2 and iv immunoglobulin (IVIg) n=2]. Cognitive dysfunction was more associated to higher SLE activity, damage and mortality. Also, psychosis was more associated to receive higher doses of oral PDN or iv MTP, to employ iv CYC and to be linked with renal SLE disease activity. On the other hand, cerebral vascular disease was more associated to receive HCQ and to have antiphospholipid antibodies. The main cause to therapy discontinuation was inefficacy and was more common in patients treated with iv CYC. Corticosteroids survival in months was higher in-group PDN <15 mg/d (97.89±16.4; IC95% 65.8–130). AZA survival in months (90±30.8; IC95% 29.6–150) was higher than others treatments for NPSLE and was more associated to use IVIg (OR 10.8, 95% CI 1.04–111, p=0.04). When the first therapy was failed, the drug as second therapy used with higher survival in months was MMF (100±60.5; IC95% 1.6–218).
Conclusions This study suggests that patients with diffuse manifestations and central nervous system involvement in NPSLE presented more activity, damage and mortality and therefore used more PDN, MTP and CYC. Among the treatments as first therapy for NPSLE with the better long-term efficacy was AZA and when it failed, MMF remained as better second therapy.
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Disclosure of Interest None declared