Anti-NS antibodies, most prevalently anti-MOG antibodies, are significantly associated with NPSLE and manifest with the distinct neurological syndrome associated with the antibody in a subgroup. Follow-up studies in large, independent cohorts will reveal whether these anti-NS antibodies could serve as a diagnostic and prognostic biomarker for NPSLE and enable tailored treatment decisions in this challenging and diverse patient cohort.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory skin disease of the anogenitalarea leading to itch, burning, sexual dysfunction and impaired quality of life. An unmet need in the context of LS is a practical, easily assessable grading scale to classify disease severity and to allow intra-and interindividual comparisons. The objectives of this study were i) to assess the prevalence and severity of 23 items proposed by a recent Delphi consensus group in patients with adult VLS. ii) to develop a clinical severity scale and, iii) to test the interrater reliability of this novel severity scale. A retrospective assessment of the prevalence and severity of 23 items in 143 patients was performed by using patient records and photo documentation to develop a novel clinical severity scale (i.e. the "Clinical Lichen Sclerosus Score" = CLISSCO) for VLS. Thereafter, the CLISSCO was validated by 16 raters. We found that the items proposed by the consensus group vary markedly in frequency and severity. Following selection of the most relevant items, the CLISSCO was developed consisting of 3 "Symptoms", 3 "Signs" and 6 "Architectural changes" rated on a 0-4 point Likert-scale. The intraclass correlation coefficient was excellent for each item, the applicability of the CLISSCO considered user-friendly by the raters. We conclude that the CLISSCO proved to be a user-friendly, reliable tool to assess disease severity in VLS. However, further studies are needed to validate its applicability and value in daily practice and clinical research.

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placebo in patients with systemic lupus erythematosus, few studies renewed interest for this molecule.We hypothesised that applying new SLE response criteria in EXPLORER study, we could show rituximab efficacy. Our objective was to reanalyze EXPLORER trial's data using four newly described SLE response criteria. Methods In our reanalysis, rituximab efficacy was assessed at week 52 using 4 criteria: SRI-4 (Systemic lupus erythematosus Responder Index) with and without a concomitant oral corticosteroid tapering objective of <10 mg at months 6 (SRI-4 with or without OCS tapering), Lupus Low Disease Activity Score (LLDAS) and BILAG-based Combined Lupus Assessment (BICLA). Results Data from all 257 patients were available. There was 234 women (91%) with a mean age of 40, 3 years among which 177 (69%) received hydroxychloroquine.At week 52, SRI-4 response rate was 27,2% in the rituximab group vs 22,7% in the placebo group (p=0.43); SRI-4 with OCS tapering was 16% in the rituximab group vs 13.6% in the placebo group (p=0.62); LLDAS was 16% in the rituximab group vs 12.5% in the placebo group (p=0.46) and BICLA was 15.4% in the rituximab group vs 15.9% in the placebo group (p=0.91).Subgroup analyses demonstrated a trend for better efficacy of rituximab compared to placebo in the subgroup of patients co-treated with methotrexate: SRI-4 of 30.6% in the rituximab group vs 12% in the placebo group (n=74, p=0.08). This trend was not found in the subgroup of patients cotreated with azathioprine or mycophenolate. In the subgroup of patients with an BILAG A/B in haematological system or vasculitis at baseline, there was a significantly higher SRI-4 response rate with rituximab: 28,6% vs 5,3% in the haematological group (p=0.047) and 39,3% vs 0% in the vasculitis group (p=0.037). Conclusions Our study confirms the results from the original EXPLORER Study. However, subgroup analysis suggests that patients with haematological or vasculitis involvement might benefit from rituximab. Efficacy in the subgroup treated with methotrexate is likely to be due to a lesser bias of concomitant immunosuppressive medication compared to azathioprine and mycophenolate.