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Reduction in cyclophosphamide cumulative dose and introduction of newer immunosuppressive drugs may reduce the malignant burden of systemic necrotising vasculitis (SNV).1 2 This study aimed to describe malignancies recorded in five randomised controlled trials in SNV conducted by the French Vasculitis Study Group and to identify predictive factors. CHUSPAN, CHUSPAN 2, WEGENT, CORTAGE and MAINRITSAN trials evaluated different therapeutic strategies, summarised in online supplementary table S1, for the treatment of newly diagnosed or relapsing SNV. Informations regarding methods and references are provided in the online supplementary materials. The primary endpoint was the occurrence of malignancy.### Supplementary data [annrheumdis-2019-216452supp001.pdf] A total of 733 patients included between 1993 and 2012 were pooled. Baseline characteristics of the population are summarised in table 1. During a 4485.9 person-years (PY) observation period, 39 (5.3%) patients developed malignancies (869.5 per 100 000 PY), including solid cancers in 34 (4.6%) cases (757.9 per 100000 PY) and haematological malignancies in 5 …

Background:There is renewed interest in the role of autoantibodies to predict outcomes in systemic sclerosis (SSc). Among the newly identified subsets, patients with limited cutaneous form (lcSSc) but anti-topoisomerase I antibodies (Scl70) positivity draw particular attention, and namely, assessing the risk of developing interstitial lung disease (ILD) —the main cause of death in SSc—to improve the management of Scl70-lcSSc patients.Objectives:We aimed to characterize patients with Scl70-lcSSc in the large multicenter European Scleroderma Trial and Research (EUSTAR) cohort.Methods:The EUSTAR database was locked in July 2019. We included all patients fulfilling 1980 ACR and/or 2013 ACR/EULAR criteria for SSc, with disease duration at database entry ≤3 yrs and known and stable skin form during the first 3 yrs. Patients with lcSSc were compared: Scl70-lcSSc (target group) vs. ACA-lcSSc and ANA-lcSSc (Step 1); and Scl70-lcSSc vs. Scl70-dcSSc (Step 2). In the ANA subgroup we included ANA+ patients with negative SSc-specific antibodies (Scl70, ACA, RNA polymerase III). In each step, we performed 5 generalized mixed models (GMM) for the risk of the new onset of ILD (defined by imaging), primary myocardial involvement (PMI), pulmonary hypertension (PH), “any severe” (ILD+PMI+PH+scleroderma renal crisis) and all-cause-mortality. An additional GMM assessed the risk of forced vital capacity (FVC) decline ≥10% vs. FVC value at ILD onset. Each GMM was adjusted for age, sex and confounders.Results:Overall, 1285 SSc patients were included: 1068 (83%) females, 860 (67%) lcSSc and 425 (33%) dcSSc. Among patients with lcSSc, 537 (62%) had ACA+, 194 (23%) Scl70+ and 129 (15%) ANA+; 425 patients had dcSSc and Scl70+. Median follow-up was similar in all 4 groups: 7.2 to 8.1 yrs.Step 1: At baseline, Scl70-lcSSc patients had significantly shorter time from Raynaud’s phenomenon (RP) to SSc onset, higher mRSS (5.8±4.8 vs. 4.3±4, p=0.001), and higher rate of articular and muscular involvement vs. ACA-lcSSc patients (Figure 1). No differences were found between Scl70-lcSSc and ANA-lcSSc comparing the aforementioned variables. ILD was more frequent in Scl70-lcSSc (46%) than in ACA-lcSSc (10%) and ANA-lcSSc (25%), as well as restrictive lung disease. GMM showed that Scl70-lcSSc carries a higher risk of ILD than both ACA-lcSSc (HR 4.55, 95%CI 3.23-6.67) and ANA-lcSSc (HR 2.17, 95%CI 1.39-3.45), with a rate of FVC decline ≥10% over time similar to the other limited forms. In Scl70-lcSSc patients the risk of “any severe” organ involvement was similar to ANA-lcSSc and higher than ACA-lcSSc (HR 1.89, 95%CI 1.40-2.50). In particular, Scl70-lcSSc shows a risk of PMI similar to ANA-lcSSc and lower than ACA-lcSSc; no differences regarding PH risk. The mortality risk in patients with Scl70-lcSSc was similar to the other limited forms’.Step 2: At baseline, time from RP to SSc onset was longer in patients with Scl70-lcSSc, with less frequent joint synovitis and tendon friction rubs vs. patients with Scl70-dcSSc. Conversely, the frequency of muscular, cardiac and pulmonary involvement was similar. The risk of ILD in Scl70-lcSSc patients was similar to Scl70-dcSSc, with a lower risk of FVC decline ≥10% over time. The risk of “any severe” involvement (HR 0.66, 95%CI 0.49-0.83), PMI and PH was lower and the mortality risk tended to be lower (HR 0.57, 95%CI 0.33-1.01, p=0.053) vs. Scl70-dcSSc.Conclusion:In our large multicenter EUSTAR cohort one quarter of lcSSc patients were Scl70+. We show a ranking for major organ involvement within lcSSc: Scl70 the most severe, ANA+ intermediate and ACA the milder form. Scl70-dcSSc patients present the most severe phenotype, and Scl70 positivity, more than the cutaneous subset, is strongly predictive of ILD, whereas other variables may influence progression. These results may provide new insight to improve the management of Scl70-lcSSc patients.Disclosure of Interests:Elisabetta Zanatta: None declared, Dörte Huscher: None declared, Paolo Airò: None declared, Alexandra Balbir-Gurman: None declared, Elise Siegert: None declared, Augusta Ortolan: None declared, Marco Matucci-Cerinic: None declared, Franco Cozzi: None declared, Gabriela Riemekasten: None declared, Anna-Maria Hoffmann-Vold: None declared, Oliver Distler Speakers bureau: has/had consultancy relationship and/or has received research funding in the area of potential treatments for systemic sclerosis and its complications from (last three years): Abbvie, Acceleron Pharma, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, ChemomAb, Corbus Pharmaceuticals, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Italfarmaco, iQone, Kymera Therapeutics, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Topadur, Target Bioscience and UCB. Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143)., Consultant of: has/had consultancy relationship and/or has received research funding in the area of potential treatments for systemic sclerosis and its complications from (last three years): Abbvie, Acceleron Pharma, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, ChemomAb, Corbus Pharmaceuticals, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Italfarmaco, iQone, Kymera Therapeutics, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Topadur, Target Bioscience and UCB. Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143)., Grant/research support from: Kymera Therapeutics, Mitsubishi Tanabe, Armando Gabrielli: None declared, Stefan Heitmann: None declared, Nicolas Hunzelmann: None declared, Carlomaurizio Montecucco: None declared, Jadranka Morovic-Vergles: None declared, Camillo Ribi: None declared, Andrea Doria: None declared, Yannick Allanore: None declared

ObjectiveTo identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc).MethodsA list of items provided by European League Against Rheumatism (EULAR) Scleroderma Trial and Research(EUSTAR) centres were subjected to a Delphi exercise among 110 experts in the field of SSc. In round 1, experts were asked to choose the items they considered as the most important for the very early diagnosis of SSc. In round 2, experts were asked to reconsider the items accepted after the first stage. In round 3, the clinical relevance of selected items and their importance as measures that would lead to an early referral process were rated using appropriateness scores.ResultsPhysicians from 85 EUSTAR centres participated in the study and provided an initial list of 121 items. After three Delphi rounds, the steering committee, with input from external experts, collapsed the 121 items into three domains containing seven items, developed as follows: skin domain (puffy fingers/puffy swollen digits turning into sclerodactily); vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). Finally, the whole assembly of EUSTAR centres ratified with a majority vote the results in a final face-to-face meeting.ConclusionThe three Delphi rounds allowed us to identify the items considered by experts as necessary for the very early diagnosis of SSc. The validation of these items to establish diagnostic criteria is currently ongoing in a prospective observational cohort.

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that impacts on patients quality of life. Treatment in SLE not only aims to decrease disease activity and damage accrual but also to improve health-related quality of life (HRQOL). Only few studies have examined the influence of detailed disease-related organ involvement on HRQOL Objectives To assess HRQOL and disease activity in a national Swiss multicentre cohort of patients with SLE (1;2). Methods Cross-sectional study on patients included in the Swiss SLE Cohort Study between April 2007 and June 2014. Inclusion criteria were: age ≥18 years, SLE defined by the American College of Rheumatology (ACR) classification criteria, written informed consent, having completed a Medical Outcome Study Short Form 36 (SF-36) at baseline and assessed for SLE disease activity at the same time. HRQOL was assessed with the mental component scale (MCS) and Physical component scale (PCS) and the eight subscales of the SF-36. Disease activity was assessed by the SLE Disease Activity Index score with the Safety of Estrogens in SLE National Assessment (SELENA-SLEDAI) modification physican9s global assessement score (PGA). Disease damage was assessed by the System Lupus Internation Collaborating Clinics/ACR Damage Index for SLE (SLICC). Results Two hundred fifty-two patients were included in the study. The median [IQR] MCS was 46.5 [34.9 – 54.1] and the median [IQR] PCS of the SF-36 was 41.1 [33.2 – 47.3]. The median [IQR] SELENA-SLEDAI score was 3 [0.75 - 8]. Univariate analysis revealed significant correlations between PCS and PGA (r=-0.18, p SELENA-SLEDAI inversely correlated with MCS (r=-0.22; p SLICC inversely correlated with PCS (-0.24; p Conclusions Health-related quality of life is low in Swiss SLE patients, particularly in those with musculoskelettal and renal involvement or diabetes. References Ribi C. et al. Swiss Med Wkly. 2014 Chizzolini C. et al. Rev Med Suisse. 2009 Disclosure of Interest None declared

Background Systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) are chronic systemic autoimmune diseases with different pathogenesis, that share susceptibility genes and both diseases can alter patients9 quality of life (1,2). Quality of life has imposed itself as an important outcome measure and the assessment of health related quality of life (HRQoL) is included in therapeutic goals and evaluation of novel treatments (3,4). Objectives To compare health-related quality of life (HRQoL) in systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) in Switzerland. Methods The primary outcome of this study was to assess and compare HRQoL in patients with SLE and RA. In this analysis, we included patients from the multicenter Swiss SLE cohort study (SSCS) and age and sex matched patients from the multicenter Swiss Clinical Quality Management Program for Rheumatoid Arthritis (SCQM) databases that were assessed for HRQoL using the SF-36 physical component scale (PCS) and mental component scale (MCS). Marginal longitudinal regression was used to identify and test predictive factors of HRQoL in the two diseases. Finally, analysis of variance was used to test HRQoL difference between both groups. Results A total of 267 patients with SLE and 267 matched patients with RA were analysed. The median [Interquartile range (IQR)] MCS and PCS scores were 46.9 [35.5-54.3] and 40.8 [33.5-47.1] in SLE and 48.8 [37.6-56.7] and 34.7 [26.8-43.0] in RA, respectively. Thus, the MCS was significantly lower in SLE patients (p=0.04), while the PCS was significantly lower in RA patients (p Conclusions While SLE and RA both affect HRQoL, patients with SLE have lower MCS whereas patients with RA have lower PCS. These alterations persist after one year of follow-up and indicate fundamental differences in the impact of disease in these two populations. References Lisnevskaia L, Murphy G, Isenberg D. Systemic lupus erythematosus. Lancet. 2014 May 29; Scott DL, Wolfe F, Huizinga TWJ. Rheumatoid arthritis. Lancet. 2010 Sep 25;376(9746):1094–108. Strand V, Levy RA, Cervera R, Petri MA, Birch H, Freimuth WW, et al. Improvements in health-related quality of life with belimumab, a B-lymphocyte stimulator-specific inhibitor, in patients with autoantibody-positive systemic lupus erythematosus from the randomised controlled BLISS trials. Ann Rheum Dis. 2014 May;73(5):838–44. Bingham CO, Weinblatt M, Han C, Gathany TA, Kim L, Lo KH, et al. The effect of intravenous golimumab on health-related quality of life in rheumatoid arthritis: 24-week results of the phase III GO-FURTHER trial. J Rheumatol. 2014 Jun;41(6):1067–76 Disclosure of Interest None declared