Proposition of a severity scale for lichen sclerosus: The "Clinical Lichen Sclerosus Score".

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.

Association of antibodies against myelin and neuronal antigens with neuroinflammation in systemic lupus erythematosus.

OBJECTIVES: To determine frequency and syndrome specificity of novel and known nervous system (NS)-directed antibodies in a large, unbiased cohort of SLE patients in the Swiss SLE Cohort Study. METHODS: This retrospective pilot study included 174 patients in a cross-sectional and 102 in a longitudinal study. Antibodies against 12 NS antigens [myelin oligodendrocyte glycoprotein (MOG), neurofascin 186 (NF186), aquaporin-4 (AQP4), N-methyl-D-aspartate receptor (subunit NR1) (NMDAR-NR1), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (subunits 1 and 2) (AMPAR1/2), gamma-aminobutyric acid B receptor (subunits B1 and B2) (GABABR1/2), glutamate decarboxylase 65 (GAD65), glycine receptor (GlyR), contactin-associated protein-like 2 (CASPR2), leucine-rich glioma-inactivated 1 (LGI1), metabotropic glutamate receptor 5 (mGluR5) and dipeptidyl-peptidase-like protein 6 (DPPX)] were screened with validated cell-based assays and correlated with clinical and diagnostic findings. RESULTS: Twenty-three of one hundred and seventy-four (13.2%) patients harboured antibodies against MOG (n = 14), NF186 (n = 6), GAD65 (n = 2), AQP4 and GlyR (n = 1). Anti-MOG antibodies were most frequently found in the cohort (8%). Thirteen of the anti-NS antibody-positive patients showed clinical symptoms of NS involvement, a subgroup of which (n = 8) resembled the syndrome associated with the antibody. Nine patients harboured antibodies without neurological symptoms and one patient was lost to follow-up. The frequency of NPSLE was significantly higher in the anti-NS antibody-positive patients (13/23, 56.5%: MOG 6/14, 42.9%; NF186 5/6, 83.3%; GAD65 2/2, 100%; AQP4/GlyR 0/1, 0%) compared with the antibody-negative cohort (21/151, 13.9%) (chi-square test, P < 0.0001). CONCLUSION: 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.