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ObjectivesThe expanded therapeutic arsenal in rheumatoid arthritis (RA) raises new clinical questions. The objective of this study is to compare the effectiveness of cycling Janus kinase inhibitors (JAKi) with switching to biologic disease-modifying antirheumatic drug (bDMARD) in patients with RA after failure to the first JAKi.MethodsThis is a nested cohort study within data pooled from an international collaboration of 17 national registries (JAK-pot collaboration). Data from patients with RA with JAKi treatment failure and who were subsequently treated with either a second JAKi or with a bDMARD were prospectively collected. Differences in drug retention rates after second treatment initiation were assessed by log-rank test and Cox regression analysis adjusting for potential confounders. Change in Clinical Disease Activity Index (CDAI) over time was estimated using a linear regression model, adjusting for confounders.Results365 cycling and 1635 switching patients were studied. Cyclers were older and received a higher number of previous bDMARDs. Both strategies showed similar observed retention rates after 2 years of follow-up. However, adjusted analysis revealed that cycling was associated with higher retention (p=0.04). Among cyclers, when the first JAKi was discontinued due to an adverse event (AE), it was more likely that the second JAKi would also be stopped due to an AE. Improvement in CDAI over time was similar in both strategies.ConclusionsAfter failing the first JAKi, cycling JAKi and switching to a bDMARD appear to have similar effectiveness. Caution is advised if an AE was the reason to stop the first JAKi.

Background:Comparing drug effectiveness in observational settings is hampered by several major threats, among them confounding and attrition bias bias (patients who stop treatment no longer contribute information, which may overestimate true drug effectiveness).Objectives:To present points to consider (PtC) when analysing and reporting comparative effectiveness with observational data in rheumatology (EULAR-funded taskforce).Methods:The task force comprises 18 experts: epidemiologists, statisticians, rheumatologists, patients, and health professionals.Results:A systematic literature review of methods currently used for comparative effectiveness research in rheumatology and a statistical simulation study were used to inform the PtC (table). Overarching principles focused on defining treatment effectiveness and promoting robust and transparent epidemiological and statistical methods increase the trustworthiness of the results.Points to considerReporting of comparative effectiveness observational studies must follow the STROBE guidelinesAuthors should prepare a statistical analysis plan in advanceTo provide a more complete picture of effectiveness, several outcomes across multiple health domains should be comparedLost to follow-up from the study sample must be reported by the exposure of interestThe proportion of patients who stop and/or change therapy over time, as well as the reasons for treatment discontinuation must be reportedCovariates should be chosen based on subject matter knowledge and model selection should be justifiedThe study baseline should be at treatment initiation and a description of how covariate measurements relate to baseline should be includedThe analysis should be based on all patients starting a treatment and not limited to patients remaining on treatment at a certain time pointWhen treatment discontinuation occurs before the time of outcome assessment, this attrition should be taken into account in the analysis.Sensitivity analyses should be undertaken to explore the influence of assumptions related to missingness, particularly in case of attritionConclusion:The increased use of real-world comparative effectiveness studies makes it imperative to reduce divergent or contradictory results due to biases. Having clear recommendations for the analysis and reporting of these studies should promote agreement of observational studies, and improve studies’ trustworthiness, which may also facilitate meta-analysis of observational data.Disclosure of Interests:Delphine Courvoisier: None declared, Kim Lauper: None declared, Sytske Anne Bergstra: None declared, Maarten de Wit Grant/research support from: Dr. de Wit reports personal fees from Ely Lilly, 2019, personal fees from Celgene, 2019, personal fees from Pfizer, 2019, personal fees from Janssen-Cilag, 2017, outside the submitted work., Consultant of: Dr. de Wit reports personal fees from Ely Lilly, 2019, personal fees from Celgene, 2019, personal fees from Pfizer, 2019, personal fees from Janssen-Cilag, 2017, outside the submitted work., Speakers bureau: Dr. de Wit reports personal fees from Ely Lilly, 2019, personal fees from Celgene, 2019, personal fees from Pfizer, 2019, personal fees from Janssen-Cilag, 2017, outside the submitted work., Bruno Fautrel Grant/research support from: AbbVie, Lilly, MSD, Pfizer, Consultant of: AbbVie, Biogen, BMS, Boehringer Ingelheim, Celgene, Lilly, Janssen, Medac MSD France, Nordic Pharma, Novartis, Pfizer, Roche, Sanofi Aventis, SOBI and UCB, Thomas Frisell: None declared, Kimme Hyrich Grant/research support from: Pfizer, UCB, BMS, Speakers bureau: Abbvie, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Joanna KEDRA: None declared, Pedro M Machado Consultant of: PMM: Abbvie, Celgene, Janssen, Lilly, MSD, Novartis, Pfizer, Roche and UCB, Speakers bureau: PMM: Abbvie, BMS, Lilly, MSD, Novartis, Pfizer, Roche and UCB, Lykke Midtbøll Ørnbjerg Grant/research support from: Novartis, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Maria Jose Santos Speakers bureau: Novartis and Pfizer, Tanja Stamm Grant/research support from: AbbVie, Roche, Consultant of: AbbVie, Sanofi Genzyme, Speakers bureau: AbbVie, Roche, Sanofi, Simon Stones Consultant of: I have been a paid consultant for Envision Pharma Group and Parexel. This does not relate to this abstract., Speakers bureau: I have been a paid speaker for Actelion and Janssen. These do not relate to this abstract., Anja Strangfeld Speakers bureau: AbbVie, BMS, Pfizer, Roche, Sanofi-Aventis, Robert B.M. Landewé Consultant of: AbbVie; AstraZeneca; Bristol-Myers Squibb; Eli Lilly & Co.; Galapagos NV; Novartis; Pfizer; UCB Pharma, Axel Finckh Grant/research support from: Pfizer: Unrestricted research grant, Eli-Lilly: Unrestricted research grant, Consultant of: Sanofi, AB2BIO, Abbvie, Pfizer, MSD, Speakers bureau: Sanofi, Pfizer, Roche, Thermo Fisher Scientific

Background:To determine the real-life 6- and 12-month secukinumab effectiveness in Europe overall, as well as stratified by prior biologic disease-modifying anti-rheumatic drug (bDMARD)/targeted synthetic (ts)DMARD use.Objectives:Real-life data from axSpA patients treated with secukinumab from 13 countries in the European Spondyloarthritis (EuroSpA) Research Collaboration Network were pooled. We calculated proportions of patients achieving Bath Ankylosing Spondylitis Disease Activity Score (BASDAI) Methods:A total of 1860 axSpA patients were included (Table 1). Overall 6/12-month secukinumab retention rates were 82%/72% and higher in bionaïve patients (Table 2, Figure). Significant differences in retention rates in-between the registries were found. Inactive disease/low-disease-activity (LDA) were achieved more often in bionaïve patients (Table 2).Table 1All patients (n=1860)b/tsDMARD naïve (n=414)1 prior b/tsDMARD (n=448)≥2 prior b/tsDMARDs (n=998)Age (years), mean (SD)47 (12)45 (12)47 (12)48 (12)Men, %57%68%58%49%Years since diagnosis, mean (SD)10 (9)8 (9)10 (9)11 (9)Current smokers, %25 %27%25%23%Patient’s global (0-100), median (IQR)70 (50-81)80 (60-90)64 (50-80)70 (50-82)Physician’s global (0-100), median (IQR)45 (25-63)64 (43-78)45 (22-60)40 (20-58)C reactive protein (mg/L), median (IQR)8 (3-25)15 (5-31)7 (3-25)6 (2-22)Erythrocyte sedimentation rate (mm/h), median (IQR)22 (9-44)30 (14-44)24 (8-45)18 (8-42)Pain (0-100), median (IQR)70 (50-81)80 (65-90)65 (49-80)70 (50-80)BASDAI, median (IQR)6.2 (4.6-7.6)6.8 (5.2-8.0)5.9 (4.2-7.2)6.1 (4.4-7.6)BASFI, median (IQR)5.5 (3.2-7.3)6.1 (3.2-7.6)4.8 (2.8-6.8)5.5 (3.3-7.2)ASDAS, median (IQR)3.6 (2.9-4.3)4.2 (3.5-4.8)3.5 (2.7-4.2)3.5 (2.8-4.2)Table 2MonthsAll patients (n=1860)b/tsDMARD naïve (n=414)1 prior b/tsDMARD (n=448)≥2 prior b/tsDMARDs (n=998)p-value*Secukinumab retention rate, % (95%CI)682% (80-84%)90% (87-93%)83% (79-86%)78% (76-81%)0.0011272% (69-74%)84% (81-88%)73% (69-78%)66% (63-69%)BASDAI Crude626373518 LUNDEX adjusted21342813 Crude1225412918 LUNDEX adjusted16311811BASDAI Crude651716040 LUNDEX adjusted40654730 Crude1251765639 LUNDEX adjusted32573623ASDAS Crude69131360.001 LUNDEX adjusted712115 Crude1211181570.002 LUNDEX adjusted713940.002ASDAS Crude6243226200.002 LUNDEX adjusted19292115 Crude1227442721 LUNDEX adjusted17331712*Comparisons between b/tsDMARD naïve, 1 prior and ≥2 prior b/tsDMARD users were performed with Kaplan-Meier with log-rank test or Chi-Square test, as appropriateConclusion:In this real-life study of 1860 patients with axSpA in 13 European countries secukinumab retention was high and significantly higher for bionaïve patients. Overall, a higher proportion of bionaïve than previous b/tsDMARD users achieved inactive disease/LDA.FigureAcknowledgments:Novartis and IQVIA for supporting the EuroSpA RCNDisclosure of Interests:Brigitte Michelsen Grant/research support from: Research support from Novartis, Consultant of: Consulting fees Novartis, Ulf Lindström: None declared, Catalin Codreanu Consultant of: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Speakers bureau: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Adrian Ciurea Consultant of: Consulting and/or speaking fees from AbbVie, Bristol-Myers Squibb, Celgene, Eli Lilly, Merck Sharp & Dohme, Novartis and Pfizer., Jakub Zavada Speakers bureau: Abbvie, UCB, Sanofi, Elli-Lilly, Novartis, Zentiva, Accord, Anne Gitte Loft Grant/research support from: Novartis, Consultant of: AbbVie, MSD, Novartis, Pfizer and UCB, Speakers bureau: AbbVie, MSD, Novartis, Pfizer and UCB, Manuel Pombo-Suarez Consultant of: Janssen, Lilly, MSD and Sanofi., Speakers bureau: Janssen, Lilly, MSD and Sanofi., Fatos Onen: None declared, Tore K. Kvien Grant/research support from: Received grants from Abbvie, Hospira/Pfizer, MSD and Roche (not relevant for this abstract)., Consultant of: Have received personal fees from Abbvie, Biogen, BMS, Celltrion, Eli Lily, Hospira/Pfizer, MSD, Novartis, Orion Pharma, Roche, Sandoz, UCB, Sanofi and Mylan (not relevant for this abstract)., Paid instructor for: Have received personal fees from Abbvie, Biogen, BMS, Celltrion, Eli Lily, Hospira/Pfizer, MSD, Novartis, Orion Pharma, Roche, Sandoz, UCB, Sanofi and Mylan (not relevant for this abstract)., Speakers bureau: Have received personal fees from Abbvie, Biogen, BMS, Celltrion, Eli Lily, Hospira/Pfizer, MSD, Novartis, Orion Pharma, Roche, Sandoz, UCB, Sanofi and Mylan (not relevant for this abstract)., Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Maria Jose Santos Speakers bureau: Novartis and Pfizer, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Anna-Mari Hokkanen: None declared, Björn Gudbjornsson Speakers bureau: Novartis and Amgen, Johan Askling Grant/research support from: JA acts or has acted as PI for agreements between Karolinska Institutet and the following entities, mainly in the context of the ARTIS national safety monitoring programme of immunomodulators in rheumatology: Abbvie, BMS, Eli Lilly, Merck, MSD, Pfizer, Roche, Samsung Bioepis, Sanofi, and UCB Pharma, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Michael Nissen Grant/research support from: Abbvie, Consultant of: Novartis, Lilly, Abbvie, Celgene and Pfizer, Speakers bureau: Novartis, Lilly, Abbvie, Celgene and Pfizer, Karel Pavelka Consultant of: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Speakers bureau: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Carlos Sánchez-Piedra: None declared, Servet Akar: None declared, Joe Sexton: None declared, Matija Tomsic: None declared, Helena Santos Speakers bureau: AbbVie, Eli-Lilly, Janssen, Pfizer, Novartis, Marco Sebastiani: None declared, Jenny Osterlund: None declared, Arni Jon Geirsson: None declared, Gareth T. Jones Grant/research support from: Pfizer, AbbVie, UCB, Celgene and GSK., Irene van der Horst-Bruinsma Grant/research support from: AbbVie, Novartis, Eli Lilly, Bristol-Myers Squibb, MSD, Pfizer, UCB Pharma, Consultant of: AbbVie, Novartis, Eli Lilly, Bristol-Myers Squibb, MSD, Pfizer, UCB Pharma, Stylianos Georgiadis Grant/research support from: Novartis, Cecilie Heegaard Brahe Grant/research support from: Novartis, Lykke Midtbøll Ørnbjerg Grant/research support from: Novartis, Merete L. Hetland Grant/research support from: BMS, MSD, AbbVie, Roche, Novartis, Biogen and Pfizer, Consultant of: Eli Lilly, Speakers bureau: Orion Pharma, Biogen, Pfizer, CellTrion, Merck and Samsung Bioepis, Mikkel Ǿstergaard Grant/research support from: AbbVie, Bristol-Myers Squibb, Celgene, Merck, and Novartis, Consultant of: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo Nordisk, Orion, Pfizer, Regeneron, Roche, Sandoz, Sanofi, and UCB, Speakers bureau: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo Nordisk, Orion, Pfizer, Regeneron, Roche, Sandoz, Sanofi, and UCB

Background:Axial spondylarthritis (axSpA) patients treated with a tumour necrosis factor inhibitor (TNFi) may receive a concomitant conventional synthetic disease-modifying anti-rheumatic drug (csDMARD), although the value of combination therapy remains unclear.Objectives:Describe the proportion and phenotype of patients with axSpA initiating their first TNFi as monotherapy compared to TNFi+csDMARD combination therapy, and to compare the 1-year TNFi retention between the two groups.Methods:Data from 13 European registries was collected. Two exposure treatment groups were defined: TNFi monotherapy at baseline (=TNFi start date) and TNFi+csDMARD combination therapy. TNFi retention rates were assessed with Kaplan-Meier curves for each country and combined. Hazard ratios (HR, 95% CI) for discontinuing the TNFi were obtained with Cox models: (i) crude; adjusted for (ii) country, and (iii) country, sex, age, calendar year, disease duration and BASDAI. Participating countries were dichotomized into two strata, depending on their 1-year retention rate being above (stratum A) or below (stratum B) the average retention rate across all countries.Results:22,196 axSpA patients were included with 34% on TNFi+csDMARD combination therapy. Baseline characteristics are presented in table 1. Overall, the crude TNFi retention rate was marginally longer in the combination therapy group (80% (79-81%)) compared to the monotherapy group (78% (77-79%)) and was primarily driven by differences in stratum B (fig. 1). TNFi retention rates varied significantly across countries (range:-11.0% to +11.3%), with a clear distinction between the 2 strata. The HRs for discontinuation over 1-year (reference=TNFi monotherapy) in the 3 models were: (i) 0.88 (0.82-0.93), (ii) 0.87 (0.82-0.92), (iii) 0.88 (0.82-0.93).Table 1Baseline characteristicsAll patients(n=22196)Country stratum ACountry stratum BTNFi mono(n=4940)csDMARD + TNFi(n=2547)TNFi mono(n=9693)csDMARD + TNFi(n=5016)Age (years), mean (SD)42.6 (12.5)43.4 (12.0)42.8 (12.2)41.6 (12.7)43.7 (12.7)Females, %41.137.738.242.044.2Disease duration (yrs), mean (SD)5.7 (8.0)6.2 (7.7)6.7 (7.4)4.9 (8.2)6.1 (8.2)Enthesitis, %50.316.733.957.859.7SJC-28, median (IQR)0 (0-1)0 (0-0)0 (0-2)0 (0-0)0 (0-2)VAS pain (0-100), mean (SD)60.9 (24.5)63.3 (26.5)67.8 (23.3)60.2 (23.4)57.2 (24.3)CRP (mg/L), median (IQR)8 (3-20)7.8 (2-20)18 (6.7-32.6)6.0 (2.7-15)8.0 (3-22)BASDAI (0-10), mean (SD)5.7 (2.1)5.7 (2.2)6.2 (2.1)5.6 (2.0)5.4 (2.2)BASFI (0-10), mean (SD)4.4 (2.5)4.4 (2.6)4.9 (2.5)4.3 (2.4)4.2 (2.9)ASDAS, mean (SD)3.5 (1.1)3.7 (1.0)4.0 (1.0)3.3 (1.0)3.3 (1.1)On Infliximab, %25.721222436Baseline csDMARD use, %-Methotrexate045063-Sulfasalazine068033-Leflunomide0801Conclusion:Considerable differences were observed across countries in the use of combination therapy and TNFi retention in axSpA patients. The overall 1-year TNFi retention was higher with csDMARD co-therapy compared to TNFi monotherapy. TNFi monotherapy had a 12-13% higher risk of treatment discontinuation.Acknowledgments:Novartis Pharma AG and IQVIAMN and BD participated equallyDisclosure of Interests:Michael Nissen Grant/research support from: Abbvie, Consultant of: Novartis, Lilly, Abbvie, Celgene and Pfizer, Speakers bureau: Novartis, Lilly, Abbvie, Celgene and Pfizer, Bénédicte Delcoigne: None declared, Daniela Di Giuseppe: None declared, Lennart T.H. Jacobsson Consultant of: AbbVie, Eli Lilly, Janssen, Novartis and Pfizer, Karen Fagerli: None declared, Anne Gitte Loft Grant/research support from: Novartis, Consultant of: AbbVie, MSD, Novartis, Pfizer and UCB, Speakers bureau: AbbVie, MSD, Novartis, Pfizer and UCB, Adrian Ciurea Consultant of: Consulting and/or speaking fees from AbbVie, Bristol-Myers Squibb, Celgene, Eli Lilly, Merck Sharp & Dohme, Novartis and Pfizer., Dan Nordström Consultant of: Abbvie, Celgene, Lilly, Novartis, Pfizer, Roche and UCB., Speakers bureau: Abbvie, Celgene, Lilly, Novartis, Pfizer, Roche and UCB., Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Maria Jose Santos Speakers bureau: Novartis and Pfizer, Manuel Pombo-Suarez Consultant of: Janssen, Lilly, MSD and Sanofi., Speakers bureau: Janssen, Lilly, MSD and Sanofi., Björn Gudbjornsson Speakers bureau: Novartis and Amgen, Heřman Mann: None declared, Nurullah Akkoc: None declared, Catalin Codreanu Consultant of: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Speakers bureau: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Irene van der Horst-Bruinsma Grant/research support from: AbbVie, Novartis, Eli Lilly, Bristol-Myers Squibb, MSD, Pfizer, UCB Pharma, Consultant of: AbbVie, Novartis, Eli Lilly, Bristol-Myers Squibb, MSD, Pfizer, UCB Pharma, Brigitte Michelsen: None declared, Gary Macfarlane: None declared, Merete L. Hetland Grant/research support from: BMS, MSD, AbbVie, Roche, Novartis, Biogen and Pfizer, Consultant of: Eli Lilly, Speakers bureau: Orion Pharma, Biogen, Pfizer, CellTrion, Merck and Samsung Bioepis, Matija Tomsic: None declared, Burkhard Moeller: None declared, Pedro Ávila-Ribeiro Grant/research support from: Novartis, Carlos Sánchez-Piedra: None declared, Heikki Relas Grant/research support from: Abbvie., Consultant of: Abbvie, Celgene, and Pfizer., Speakers bureau: Abbvie, Celgene, and Pfizer., Arni Jon Geirsson: None declared, Lucie Nekvindova: None declared, Gozde Yildirim Cetin Speakers bureau: AbbVie, Novartis, Pfizer, Roche, UCB, MSD, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Niels Steen Krogh: None declared, Johan Askling Grant/research support from: JA acts or has acted as PI for agreements between Karolinska Institutet and the following entities, mainly in the context of the ARTIS national safety monitoring programme of immunomodulators in rheumatology: Abbvie, BMS, Eli Lilly, Merck, MSD, Pfizer, Roche, Samsung Bioepis, Sanofi, and UCB Pharma, Bente Glintborg Grant/research support from: Grants from Pfizer, Biogen and Abbvie, Ulf Lindström: None declared

Background:Adult Onset Still’s disease (AOSD) is an uncommon systemic inflammatory disease.Objectives:To determine for the first time the incidence rate of AOSD in our population.Methods:We retrospectively collected AOSD cases diagnosed between 1 January 2010 and 31 December 2019 at our secondary/tertiary rheumatology centre, which is the only referral centre for an average population of 704,000 adults. AOSD cases were identified by searching the electronic medical database both for ICD-10 code M06.1 and a full text search for »AOSD«. Patients’ records were analyzed and descriptive statistics was used to describe our study group. The adult population was obtained from the national statistics institute database. The annual incidence rate for AOSD was calculated.Results:During the 10-year observation period we identified 22 incipient AOSD cases. All 22 cases fulfilled Yamaguchi classification criteria for AOSD1. Five cases were excluded from analyses since they were referred to our department from regions served by other secondary/tertiary centres. Hence, we finally analyzed 17 AOSD cases (11 females; median (IQR) age 38.9 (29.9; 56.5) years, range 20-71 years), resulting in the average annual incidence rate of 2.4 (95%CI 1.5-3.8) cases per 106adults. Age specific incidence rate of AOSD is presented in Figure 1. Clinical characteristics of AOSD cases at presentation are shown in Table 1. AOSD was complicated with macrophage activating syndrome in 4/17 (23.5%) cases, and with pulmonary hypertension in one case. Patients were followed for a median (IQR) 31 (20; 58) months. Twelve (70.5%), 2 (11.8%), and 3 (17.6) patients had monophasic, relapsing, and chronic disease course, respectively.Table 1.Clinical characteristics of AOSD at presentationCharacteristicAOSD (%)CharacteristicAOSD (%)Female gender64.7Lung infiltrates23.5Age*38.9 (29.9;56.5)Pericardial effusion23.5Fever94.1Abdominal pain17.6Weight loss64.7Lymphadenopathy52.9Skin rash76.5Splenomegaly41.2Throat pain88.2Hepatomegaly17.6Arthralgia/Arthritis88.2/47.1Leukocytosis88.2Myalgia29.4Elevated AST/ALT88.2Pleural effusion23.5Ferritin >1000ng/ml94.1Legend: *median (IQR);Figure 1.Age specific incidence rate of AOSDConclusion:AOSD is rare in our population, with an average annual incidence rate of 2.4 cases per 106adults.References:[1]Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19(3):424-30.Acknowledgments -Disclosure of Interests:ALOJZIJA HOCEVAR: None declared, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Monika Krosel: None declared, Martina Plešivčnik Novljan: None declared, Sonja Praprotnik: None declared, Matija Tomsic: None declared

Background:In many countries, JAK-inhibitors (JAKi) have only recently been approved as treatment for patients with rheumatoid arthritis (RA).Objectives:To evaluate the effectiveness of JAKi compared to bDMARDs in RA patients in the real-world population in an international collaboration of registers (the “JAK-pot” collaboration).Methods:Patients initiating either JAKi, TNFi, IL-6i or abatacept (ABA) during a time period when JAKi were available in each country (19 registers, Table) were included. We compared the effectiveness of JAKi and bDMARDs in terms of retention using crude and adjusted survival analysis. Missing covariates were imputed using multiple imputation.Results:Among 25521 included patients, 6063 initiated a JAKi, 13879 a TNFi, 2348 ABA, and 3231 an IL-6i. Patients were on average 55 years old, with a mean disease duration 10 years, mostly seropositive (67%), female (77%) and with moderate disease activity at treatment initiation. The main reason of stopping treatment was ineffectiveness (49%), followed by adverse events (21%). Patients on JAKi were treated more often as monotherapy, had higher CRP and disease activity at baseline and had experienced more previous ts/bDMARDs. Crude median retention was 1.4 (95% CI 1.2-1.5) years for JAKi, 1.6 (1.6-1.7) for TNFi, 1.5 (1.3-1.7) for IL6i and 1.1 (1.0-1.3) for ABA. After adjustment, the hazard ratio (HR) for discontinuation tended to be lower for JAKi (HR 0.86 (0.65-1.13)) compared to TNFi, but comparable for ABA (1.02 (0.94-1.10)) and IL6i (0.99 (0.88-1.10)) (Figure 1). HRs differed notably between countries (Figure 2).Table 1.RegistersCountry, registerNJAKi, n (%)Austria, BIOREG*Belgium, TARDIS62882113 (33.6)Canada, RHUMADATA528114 (21.6)Czech Republic, ATTRA374253 (67.6)Denmark, DANBIO4721506 (10.7)Finland, ROB-FIN807234 (29.0)Germany, RABBIT*Italy, GISEA757250 (33.0)Israel, I-RECORD40094 (23.5)Netherlands, METEOR16424 (0.2)Norway, NOR-DMARD50799 (19.5)Portugal, REUMA.PT79744 (5.5)Romania, RRBR593328 (55.3)Russia, ARBITER526483 (91.8)Slovenia, BIORX.SI583146 (25.0)Spain, BIOBADASER781139 (17.8)Switzerland, SCQM2956796 (26.9)Turkey, TURKBIO2150397 (18.5)UK, BSRBR111163 (5.7)*Registers planning to participate in future studies but not included yetConclusion:The adjusted overall drug retention of JAKi tended to be higher than for TNFi, with large variation between countries. Other measures of effectiveness, such as the evaluation of CDAI remission and low disease activity are planned to shape a more comprehensive picture of JAKi effectiveness in the real world.Disclosure of Interests:Kim Lauper: None declared, Denis Mongin: None declared, Sytske Anne Bergstra: None declared, Denis Choquette Grant/research support from: Rhumadata is supported by grants from Pfizer, Amgen, Abbvie, Gylead, BMS, Novartis, Sandoz, eli Lilly,, Consultant of: Pfizer, Amgen, Abbvie, Gylead, BMS, Novartis, Sandoz, eli Lilly,, Speakers bureau: Pfizer, Amgen, Abbvie, Gylead, BMS, Novartis, Sandoz, eli Lilly,, Catalin Codreanu Consultant of: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Speakers bureau: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Diederik De Cock: None declared, Lene Dreyer: None declared, Ori Elkayam Speakers bureau: AbbVie, BMS, Pfizer, Roche, Sanofi-Aventis, Novartis, Jansen, Kimme Hyrich Grant/research support from: Pfizer, UCB, BMS, Speakers bureau: Abbvie, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Nevsun Inanc: None declared, Eirik kristianslund: None declared, Tore K. Kvien Grant/research support from: Received grants from Abbvie, Hospira/Pfizer, MSD and Roche (not relevant for this abstract)., Consultant of: Have received personal fees from Abbvie, Biogen, BMS, Celltrion, Eli Lily, Hospira/Pfizer, MSD, Novartis, Orion Pharma, Roche, Sandoz, UCB, Sanofi and Mylan (not relevant for this abstract)., Paid instructor for: Have received personal fees from Abbvie, Biogen, BMS, Celltrion, Eli Lily, Hospira/Pfizer, MSD, Novartis, Orion Pharma, Roche, Sandoz, UCB, Sanofi and Mylan (not relevant for this abstract)., Speakers bureau: Have received personal fees from Abbvie, Biogen, BMS, Celltrion, Eli Lily, Hospira/Pfizer, MSD, Novartis, Orion Pharma, Roche, Sandoz, UCB, Sanofi and Mylan (not relevant for this abstract)., Burkhard Leeb Grant/research support from: chairman of BioReg, Consultant of: AbbVie, Pfizer, Roche, Lilly, Grünenthal, Gebro,, Paid instructor for: Lilly, Biogen, Speakers bureau: Biogen, Lilly, Pfizer, Grünenthal, Astropharma,, Galina Lukina Speakers bureau: Novartis, Pfizer, UCB, Abbvie, Biocad, MSD, Roche, Dan Nordström Consultant of: Abbvie, Celgene, Lilly, Novartis, Pfizer, Roche and UCB., Speakers bureau: Abbvie, Celgene, Lilly, Novartis, Pfizer, Roche and UCB., Karel Pavelka Consultant of: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Speakers bureau: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Manuel Pombo-Suarez Consultant of: Janssen, Lilly, MSD and Sanofi., Speakers bureau: Janssen, Lilly, MSD and Sanofi., Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Maria Jose Santos Speakers bureau: Novartis and Pfizer, Anja Strangfeld Speakers bureau: AbbVie, BMS, Pfizer, Roche, Sanofi-Aventis, Delphine Courvoisier: None declared, Axel Finckh Grant/research support from: Pfizer: Unrestricted research grant, Eli-Lilly: Unrestricted research grant, Consultant of: Sanofi, AB2BIO, Abbvie, Pfizer, MSD, Speakers bureau: Sanofi, Pfizer, Roche, Thermo Fisher Scientific

Background:Anticardiolipin antibodies (aCL) can be detected in newly diagnosed GCA as reactive antibodies to endothelial lesions. Their prognostic role, as a marker of disease activity, has not been extensively studied in GCA.Objectives:Our aim was to determine whether aCL IgG might represent laboratory marker of active GCA.Methods:We included patients with new clinical diagnosis of GCA supported by histology or imaging between September 2011 and July 2019, who completed at least a 48-week follow-up at our secondary/tertiary rheumatology center. Follow up visits with predetermined clinical and laboratory tests, including aCL IgG, were performed 12, 24, 48, and 96 weeks after diagnosis. GCA relapse was defined as worsening or new disease activity after initial remission. Other reasons for the disease-related symptoms, elevated inflammatory markers (C reactive protein or erythrocyte sedimentation rate) had to be excluded. aCL IgG were determined in the patients’ sera samples at baseline and at follow up visits, using an in-house solid phase enzyme-linked immunosorbent assay1. A value above the 99th percentile of the healthy control population was taken as significant.Results:During the observation period we identified 288 newly diagnosed GCA patients. Two hundred and twelve GCA patients (66.5% females, median (IQR) age 73.9 (67.0–78.7) years) fulfilled the study inclusion criterion, among them 145 patients completed the 96-week follow up visit. At baseline, 129/212 (60.8%) GCA patients had positive aCL IgG. During in total 781 follow up visits, we recorded 77 (9.9%) episodes of active/relapsing GCA (clinical, laboratory or combined in 4 (5.2%), 48 (62.3%), 25 (32.5%) episodes, respectively). aCL IgG were present at 155/781 (19.8%) measurements (at 24/77 episodes of relapsing/active and 131/573 episodes of quiescent GCA). The correlation between active/relapsing GCA and aCL IgG positivity was only weekly positive (r coefficient=0.094; p= 0.015).Conclusion:The role of aCL IgG as a biomarker for GCA activity seems to be rather limited.References:[1]Božič B, et al. Int Arch Allerg Immunol. 1997; 112:19-26. doi.org/ 10.1159/000237426Disclosure of Interests:None declared

Background:IgA vasculitis (IgAV) could be limited to skin or evolve into a systemic disease, affecting characteristically joints, gastrointestinal tract and/or kidneys.Objectives:We aimed to look for differences between adult IgAV patients with disease limited to skin compared to systemic IgAV.Methods:Medical records of histologically proven adult IgAV cases, diagnosed between January 2010 and December 2020 at our secondary/tertiary rheumatology centre were analyzed.Results:During the 132-month observation period we identified 328 new IgAV cases (59.5% males, median (IQR) age 64.3 (45.1; 76.1) years). Ninety-four (40.2%) patients had skin limited disease, and the rest systemic IgAV.Clinical differences between skin limited and systemic adult IgAV are presented in table 1. Adults with IgAV limited to skin were significantly older, had less commonly skin lesions above the waistline and a lower level of C reactive protein compared to patients with a systemic disease. There were no differences in the frequency of skin necroses between the compared IgAV subgroups. The frequency of potential vasculitis triggers (prior infections, new medications, malignancy) was similar between the compared subgroups.Table 1.Clinical characteristics of IgA vasculitis patients with skin limited and systemic diseaseClinical characteristicsSkin limited IgAV (94)Systemic IgAV (234)P valueMale gender (%)54.361.50.263Age (years)*68.0 (55.0-80.5)61.5 (41.7-75.8)0.007Current smoker (%)13.821.80.123Antecedent infection (%)28.733.80.434New medication23.423.51.0History of cancer12.810.70.569Symptom duration (days)*7 (5-21)8 (5-14)0.756Purpura above waistline36.255.60.002Skin necroses (%)52.145.70.329ESR /mm/h) *32 (18-52)34 (17-53)0.873CRP (g/l) *13.5 (1-32)30 (11-68)Elevated serum IgA (%)50.649.10.892Legend: * median and IQR;Follow up data were available for 250 (76.2%) patients. During the follow up of median (IQR) 12.5 (6.8 – 22.4) months 35 patients relapsed (13/70 (18.6%) with skin limited IgAV and 22/180 (12.2%) with systemic IgAV, p= 0.224).Conclusion:Skin limited IgAV was associated with older age and less extensive skin puprura in adults. However, relapses of purpura were as common as in systemic IgAV.Disclosure of Interests:None declared

Background:Knowledge of changes over time in baseline characteristics and tumor necrosis factor inhibitor (TNFi) response in bionaïve psoriatic arthritis (PsA) patients treated in routine care is limited.Objectives:To investigate secular trends in baseline characteristics and retention, remission and response rates in PsA patients initiating a first TNFi.Methods:Prospectively collected data on bionaïve PsA patients starting TNFi in routine care from 15 European countries were pooled. According to year of TNFi initiation, three groups were defined a priori based on bDMARD availability: Group A (1999–2008), Group B (2009–2014) and Group C (2015–2018).Retention rates (Kaplan-Meier), crude and LUNDEX adjusted1 remission (Disease Activity Score (DAS28) Results:A total of 17453 PsA patients were included (4069, 7551 and 5833 in groups A, B and C).Patients in group A were older and had longer disease duration compared to B and C. Retention rates at 6, 12 and 24 months were highest in group A (88%/77%/64%) but differed little between B (83%/69%/55%) and C (84%/70%/56%).Baseline disease activity was higher in group A than in B and C (DAS28: 4.6/4.3/4.0, DAPSA28: 29.9/25.7/24.0, CDAI: 21.8/20.0/18.6), and this persisted at 6 and 12 months. Crude and LUNDEX adjusted remission rates at 6 and 12 months tended to be lowest in group A, although crude/LUNDEX adjusted ACR50 response rates at all time points were highest in group A. At 24 months, disease activity and remission rates were similar in the three groups (Table).Table 1.Secular trends in baseline characteristics, treatment retention, remission and response rates in European PsA patients initiating a 1st TNFiBaseline characteristicsGroup A(1999–2008)Group B(2009–2014)Group C(2015–2018)Age, median (IQR)62 (54–72)58 (49–67)54 (45–62)Male, %514847Years since diagnosis, median (IQR)5 (2–10)3 (1–9)3 (1–8)Smokers, %161717DAS28, median (IQR)4.6 (3.7–5.3)4.3 (3.4–5.1)4.0 (3.2–4.8)DAPSA28, median (IQR)29.9 (19.3–41.8)25.7 (17.2–38.1)24.0 (16.1–35.5)CDAI, median (IQR)21.8 (14.0–31.1)20.0 (13.0–29.0)18.6 (12.7–26.1)TNFi drug, % (Adalimumab / Etanercept / Infliximab / Certolizumab / Golimumab)27 / 43 / 30 / 0 / 036 / 31 / 14 / 5 / 1421 / 40 / 21 / 8 / 10Follow up6 months12 months24 monthsGr AGr BGr CGr AGr BGr CGr AGr BGr CRetention rates, % (95% CI)88 (87–89)83 (82–84)84 (83–85)79 (78–80)72 (71–73)72 (71–73)68 (67–69)60 (59–61)60 (59–62)DAS28, median (IQR)2.7 (1.9–3.6)2.4 (1.7–3.4)2.3 (1.7–3.2)2.5 (1.8–3.4)2.2 (1.6–3.1)2.1 (1.6–2.9)2.1 (1.6–3.1)2.0 (1.6–2.9)1.9 (1.5–2.6)DAPSA28, median (IQR)10.6 (4.8–20.0)9.5 (3.9–18.3)8.7 (3.6–15.9)9.1 (4.1–17.8)7.7 (3.1–15.4)7.6 (2.9–14.4)6.7 (2.7–13.7)6.6 (2.7–13.5)5.9 (2.4–11.8)CDAI, median (IQR)7.8 (3.0–15.2)8.0 (3.0–15.0)6.4 (2.6–12.2)6.4 (2.5–13.0)6.2 (2.5–12.1)5.8 (2.2–11.4)5.0 (2.0–11.0)5.5 (2.0–11.2)5.0 (2.0–9.0)DAS28 remission, %, c/L47 / 4255 / 4661 / 5153 / 4362 / 4566 / 4864 / 4268 / 3775 / 41DAPSA28 remission, %, c/L22 / 1926 / 2228 / 2325 / 2031 / 2232 / 2336 / 2334 / 1938 / 21CDAI remission, %, c/L23 / 2123 / 1926 / 2227 / 2127 / 2029 / 2134 / 2231 / 1735 / 19ACR50 response, %, c/L26 / 2322 / 1824 / 2027 / 2223 / 1721 / 1523 / 1518 / 1014 / 8Gr, Group; c/L, crude/LUNDEX.Conclusion:Over the past 20 years, patient age, disease duration and disease activity level at the start of the first TNFi in PsA patients have decreased. Furthermore, TNFi retention rates have decreased while remission rates have increased, especially remission rates within the first year of treatment. These findings may reflect a greater awareness of early diagnosis in PsA patients, a lowered threshold for initiating TNFi and the possibility for earlier switching in patients with inadequate treatment response.References:[1]Arthritis Rheum 2006; 54: 600-6.Acknowledgements:Novartis Pharma AG and IQVIA for supporting the EuroSpA Research Collaboration Network.Disclosure of Interests:Sara Nysom Christiansen Speakers bureau: BMS and GE, Grant/research support from: Novartis, Lykke Midtbøll Ørnbjerg Grant/research support from: Novartis, Simon Horskjær Rasmussen: None declared, Anne Gitte Loft Speakers bureau: AbbVie, Janssen, Lilly, MSD, Novartis, Pfizer, UCB, Consultant of: AbbVie, Janssen, Lilly, MSD, Novartis, Pfizer, UCB, Grant/research support from: Novartis, Johan K Wallman Consultant of: Celgene, Eli Lilly, Novartis, Florenzo Iannone Speakers bureau: Abbvie, MSD, Novartis, Pfizer and BMS, Brigitte Michelsen Consultant of: Novartis, Grant/research support from: Novartis, Michael J. Nissen Speakers bureau: Novartis, Eli Lilly, Celgene, and Pfizer, Consultant of: Novartis, Eli Lilly, Celgene, and Pfizer, Jakub Zavada: None declared, Maria Jose Santos Speakers bureau: AbbVie, Novartis, Pfizer, Manuel Pombo-Suarez: None declared, Kari Eklund: None declared, Matija Tomsic Speakers bureau: Abbvie, Amgen, Biogen, Medis, MSD, Novartis, Pfizer, Consultant of: Abbvie, Amgen, Biogen, Medis, MSD, Novartis, Pfizer, Björn Gudbjornsson Speakers bureau: Amgen and Novartis, İsmail Sari: None declared, Catalin Codreanu Speakers bureau: AbbVie, Amgen, Egis, Novartis, Pfizer, UCB, Grant/research support from: AbbVie, Amgen, Egis, Novartis, Pfizer, UCB, Daniela Di Giuseppe: None declared, Bente Glintborg Grant/research support from: Pfizer, Biogen, AbbVie, Marco Sebastiani: None declared, Karen Minde Fagerli: None declared, Burkhard Moeller: None declared, Karel Pavelka Speakers bureau: AbbVie, Roche, MSD, UCB, Pfizer, Novartis, Egis, Gilead, Eli Lilly, Consultant of: AbbVie, Roche, MSD, UCB, Pfizer, Novartis, Egis, Gilead, Eli Lilly, Anabela Barcelos: None declared, Carlos Sánchez-Piedra: None declared, Heikki Relas: None declared, Ziga Rotar Speakers bureau: Abbvie, Amgen, Biogen, Medis, MSD, Novartis, Pfizer, Consultant of: Abbvie, Amgen, Biogen, Medis, MSD, Novartis, Pfizer, Thorvardur Love: None declared, Servet Akar: None declared, Ruxandra Ionescu Speakers bureau: Abbvie, Amgen, Boehringer-Ingelheim Eli-Lilly,Novartis, Pfizer, Sandoz, UCB, Gary Macfarlane Grant/research support from: GlaxoSmithKline, Marleen G.H. van de Sande: None declared, Merete L. Hetland Speakers bureau: Abbvie, Biogen, BMS, Celltrion, Eli Lilly, Janssen Biologics B.V, Lundbeck Fonden, MSD, Pfizer, Roche, Samsung Biopies, Sandoz, Novartis., Mikkel Østergaard Speakers bureau: AbbVie, BMS, Boehringer-Ingelheim, Celgene, Eli-Lilly, Centocor, GSK, Hospira, Janssen, Merck, Mundipharma, Novartis, Novo, Orion, Pfizer, Regeneron, Schering-Plough, Roche, Takeda, UCB and Wyeth, Consultant of: AbbVie, BMS, Boehringer-Ingelheim, Celgene, Eli-Lilly, Centocor, GSK, Hospira, Janssen, Merck, Mundipharma, Novartis, Novo, Orion, Pfizer, Regeneron, Schering-Plough, Roche, Takeda, UCB and Wyeth

Background:Arthritis is one of the main clinical features of IgA vasculitis (IgAV). Joint involvement represents the second most common manifestation in childhood IgAV with the predilection for the knees and ankles.Objectives:We aimed to describe the characteristics of arthritis in adult IgAV cohort.Methods:We analysed medical records of histologically proven adult IgAV cases, diagnosed between January 2010 and December 2020 at our secondary/tertiary rheumatology centre. The frequency, temporal occurrence and the localization of arthritis was recorded. In addition, we searched for potential differences in other IgAV features between patients with and without arthritis.Results:During the 132-month observation period we identified 328 new IgAV cases (59.5% males, median (IQR) age 64.3 (45.1; 76.1) years). Forty-eight (14.6%) patients developed arthritis. Arthritis was the first IgAV manifestation in 16 (4.9%) patients. Arthritis was mono-, oligo- and poly- articular (involving up to 15 joints) in 13 (4.0%), 25 (7.6%) and 10 (3.0%) patients, respectively. Arthritis was most common in wrists and ankles (each in 18 (37.5%) patients); metacarpophalangeal joints and knees (each in 11 (22.9%)); proximal interphalangeal joints (9 (18.8%)); elbows (8 (16.7%)) and metatarsophalangeal joints (4 (8.3%)). Clinical differences between IgAV patients with and without arthritis are presented in table 1. Patients with arthritis were significantly younger, more commonly developed gastrointestinal tract involvement compared to those without arthritis. Arthritis remitted in all with immunomodulatory treatment (given predominantly for necrotic skin purpura or visceral involvement). Follow up (FU) data accessible for 42/48 (87.5%) patients with arthritis showed that IgAV relapsed in 10 (23.8%) patients during a median (IQR) 24.5 (12.9; 40.7) month FU. Relapses were limited to skin and/or kidneys, there were no relapses of arthritis.Table 1.Clinical characteristics of IgA vasculitis patients with and without arthritisClinical characteristicsArthritis IgAV(48)Non-arthritis IgAV(280)P valueMale gender (%)56.360.00.636Age (years)*49.9 (36.1-66.9)65.4 (48.4;77.5)Ever smoker (%)50.043.60.435Prior infection (%)43.830.40.094Generalized purpura50.050.01.0Skin necroses (%)35.449.60.085GI involvement (%)47.925.00.002Renal involvement (%)47.946.81.0Elevated serum IgA (%)33.3 (11/33)52.0 (115/221)0.061Legend: * median and IQR; GI gastrointestinal;Conclusion:Arthritis in adult IgAV was frequently oligoarticular, involved large and small joints of both upper and lower extremities, and was not prone to chronic course and recurrence. In addition, we found an association between arthritis and gastrointestinal tract involvement.Disclosure of Interests:None declared

Background:Comparative effectiveness studies using observational data are increasingly used. Despite their high potential for bias, there are no detailed recommendations on how these studies should best be analysed and reported in rheumatology.Objectives:To conduct a systematic literature review of comparative effectiveness research in rheumatology to inform the EULAR task force developing points to consider when analysing and reporting comparative effectiveness research with observational data.Methods:All original articles comparing drug effectiveness in longitudinal observational studies of ≥100 patients published in key rheumatology journals (Scientific Citation Index > 2) between 1.01.2008 and 25.03.2019 available in Ovid MEDLINE® were included. Titles and abstracts were screened by two reviewers for the first 1000 abstracts and independently checked to ensure sufficient agreement has been reached. The main information extracted included the types of outcomes used to assess effectiveness, and the types of analyses performed, focusing particularly on confounding and attrition.Results:9969 abstracts were screened, with 218 articles proceeding to full-text extraction (Figure 1), representing a number of rheumatic and musculoskeletal diseases. Agreement between the two reviewers for the first 1000 abstracts was 92.7% with a kappa of 0.6. The majority of the studies used several outcomes to evaluate effectiveness (Figure 2A). Most of the studies did not explain how they addressed missing data on the covariates (70%) (Figure 2B). When addressed (30%), 44% used complete case analysis and 10% last observation carried forward (LOCF). 25% of studies did not adjust for confounding factors and there was no clear correlation between the number of factors used to adjust and the number of participants in the studies. An important number of studies selected covariates using bivariate screening and/or stepwise selection. 86% of the studies did not acknowledge attrition (Figure 2C). When trying to correct for attrition (14%), 38% used non-responder (NR) imputation, 24% used LUNDEX1, a form of NR imputation, and 21% LOCF.Conclusion:Most of studies used multiple outcomes. However, the vast majority did not acknowledge missing data and attrition, and a quarter did not adjust for any confounding factors. Moreover, when attempting to account for attrition, several studies used methods which potentially increase bias (LOCF, complete case analysis, bivariate screening…). This systematic review confirms the need for the development of recommendations for the assessment and reporting of comparative drug effectiveness in observational data in rheumatology.References:[1]Kristensen et al. A&R. 2006 Feb;54(2):600-6.Acknowledgments:Support of the Standing Committee on Epidemiology and Health Services ResearchDisclosure of Interests:Kim Lauper: None declared, Joanna KEDRA: None declared, Maarten de Wit Grant/research support from: Dr. de Wit reports personal fees from Ely Lilly, 2019, personal fees from Celgene, 2019, personal fees from Pfizer, 2019, personal fees from Janssen-Cilag, 2017, outside the submitted work., Consultant of: Dr. de Wit reports personal fees from Ely Lilly, 2019, personal fees from Celgene, 2019, personal fees from Pfizer, 2019, personal fees from Janssen-Cilag, 2017, outside the submitted work., Speakers bureau: Dr. de Wit reports personal fees from Ely Lilly, 2019, personal fees from Celgene, 2019, personal fees from Pfizer, 2019, personal fees from Janssen-Cilag, 2017, outside the submitted work., Bruno Fautrel Grant/research support from: AbbVie, Lilly, MSD, Pfizer, Consultant of: AbbVie, Biogen, BMS, Boehringer Ingelheim, Celgene, Lilly, Janssen, Medac MSD France, Nordic Pharma, Novartis, Pfizer, Roche, Sanofi Aventis, SOBI and UCB, Thomas Frisell: None declared, Kimme Hyrich Grant/research support from: Pfizer, UCB, BMS, Speakers bureau: Abbvie, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Pedro M Machado Consultant of: PMM: Abbvie, Celgene, Janssen, Lilly, MSD, Novartis, Pfizer, Roche and UCB, Speakers bureau: PMM: Abbvie, BMS, Lilly, MSD, Novartis, Pfizer, Roche and UCB, Lykke Midtbøll Ørnbjerg Grant/research support from: Novartis, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Maria Jose Santos Speakers bureau: Novartis and Pfizer, Tanja Stamm Grant/research support from: AbbVie, Roche, Consultant of: AbbVie, Sanofi Genzyme, Speakers bureau: AbbVie, Roche, Sanofi, Simon Stones Consultant of: I have been a paid consultant for Envision Pharma Group and Parexel. This does not relate to this abstract., Speakers bureau: I have been a paid speaker for Actelion and Janssen. These do not relate to this abstract., Anja Strangfeld Speakers bureau: AbbVie, BMS, Pfizer, Roche, Sanofi-Aventis, Robert B.M. Landewé Consultant of: AbbVie; AstraZeneca; Bristol-Myers Squibb; Eli Lilly & Co.; Galapagos NV; Novartis; Pfizer; UCB Pharma, Axel Finckh Grant/research support from: Pfizer: Unrestricted research grant, Eli-Lilly: Unrestricted research grant, Consultant of: Sanofi, AB2BIO, Abbvie, Pfizer, MSD, Speakers bureau: Sanofi, Pfizer, Roche, Thermo Fisher Scientific, Sytske Anne Bergstra: None declared, Delphine Courvoisier: None declared

Background:Objectives:To evaluate the role of serum IgA level as a biomarker in adult IgA vasculitis (IgAV).Methods:This prospective study included large cohort of histologically proven adult IgAV cases diagnosed between January 2013 and December 2019 at our secondary/tertiary rheumatology centre. All patients underwent a detailed clinical evaluation and laboratory workup. Patients were then stratified based on baseline serum IgA level into two groups (elevated serum IgA vs. normal serum IgA), and clinical features were compared between the two groups. Next we used multivariable logistic regression analysis to determine factors predicting gastrointestinal (GI) or renal involvement in adult IgAV.Results:During the 84-month observation period, we identified 227 incipient adult IgAV cases (60.6% males, median (interquartile range) age 64 (47–76) years, 44 (19.4%) current smokers). One hundred and eleven (48.9%) patients had elevated serum IgA level at baseline, the rest had normal IgA level. None of the patients had subnormal serum IgA level. Skin involvement, constitutional symptoms, arthritis, GI tract and renal involvement developed in 227 (100%), 32 (14.1%), 30 (13.2%), 62 (27.3%), and 93 (41.0%) patients, respectively. Patients with elevated serum IgA less frequently developed constitutional symptoms (p=0.036) and GI tract involvement (p=0.017), but had more commonly renal involvement (p Table 1.Clinical characteristic of IgAV patients with and without elevated serum IgACharacteristicAll IgAVElevated s++++IgANormal sIgAp valueNumber of patients (%)227111 (48.9)116 (51.1)–Males (%)60.867.654.30.043Age (years)#64 (47-76)58 (37-74)66 (55-77)0.289Prior infection (%)32.226.137.90.065Constitutional symptoms (%)14.19.019.00.036Generalized purpura* (%)52.055.049.10.426Necrotic purpura (%)46.353.239.70.046Arthritis (%)13.29.017.20.079GI involvement (%)27.319.834.50.017Renal involvement (%)41.053.229.3Legend: # median (IQR); GI gastrointestinal; * above the waistline;Table 2.Risk factors of GI and renal involvement, multiple logistic regressionCharacteristicGI involvementRenal involvementOR (95%CI)OR (95%CI)Age0.98 (0.96-1.0)1.02 (1.00–1.04)Current smoking–3.32 (1.56–7.07)Generalized purpura*5.86 (2.82–12.16)2.03 (1.13–3.66)↑ serum IgA0.47 (0.23–0.95)2.71 (1.48–4.96)NLR >3.53.37 (1.59–7.12)2.24 (1.19–4.23)Legend: * purpura above the waistline; NLR neutrophil to lymphocyte ratioConclusion:Serum IgA level might be a useful biomarker in IgA vasculitis, identifying patients at risk for visceral (GI and renal) involvement.Disclosure of Interests:ALOJZIJA HOCEVAR: None declared, Matija Tomsic: None declared, Vesna Jurcic: None declared, Katja Perdan-Pirkmajer: None declared, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi.

Background:Recently, an association between anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and interstitial lung disease (ILD) has been uncovered.Objectives:To determine the rate of ILD in our prospective AAV patient cohort and to compare clinical characteristics of AAV patients with and without associated ILD.Methods:We retrospectively analysed medical records of prospectively diagnosed and followed AAV patients at our secondary/tertiary rheumatology centre between January 2010 and December 2019. The diagnosis of ILD was based on lung HRCT findings.Results:During the 10-year observation, we identified 94 incipient AAV patients (46 had granulomatosis with polyangiitis, and 48 microscopic polyangiitis). Thirteen (13.8%) patients had ILD (ILD-AAV group). 12/13 had usual interstitial pneumonia (UIP) pattern and 1/13 non-specific fibrosis on HRCT. ILD was diagnosed in tandem with AAV in 9/13 patients, and 9 months to 5 years prior to AAV in 4/17 patients. Characteristics of ILD-AAV, and non-ILD-AAV groups are presented in Table 1. ILD-AAV patients more commonly reported of weight loss, less frequently had ENT involvement, and were predominantly a-MPO ANCA positive (92.3%). Follow up data were available for 85 AAV patients (90.4%; 13 ILD-AAV and 72 non-ILD-AAV). During the median (IQR) follow up of 22.1 (4.8; 50.0) months, 5/13 (38.5%) ILD-AAV patients died, compared to 6 (8.3%) deaths registered in non-ILD-AAV group during 26.4 (11.6; 70.0) months of follow up. The crude mortality rate evaluated by Cox proportional hazards regression was significantly higher for AAV-ILD group (HR 5.6 (95%CI 1.7-18.7), p=0.005).Table 1.Clinical characteristics of ILD-AAV and non-ILD-AAV groupCharacteristicILD-AAV (13)non-ILD- AAV (81)pCharacteristicILD-AAV (13)non-ILD-AAV (81)pFemale46.264.20.234ENT060.5Age*76 (67;77)66 (55;77)0.174Heart07.40.591Smoking61.539.50.226GI tract15.47.40.305Fever61.553.10.766Kidney53.863.00.552Weight loss84.651.90.035PNS38.529.60.531Arthritis15.414.81.0CNS02.51.0Myalgia15.427.20.504ANCA10091.40.588Skin7.719.80.451a-MPO92.344.40.002Eye024.70.063a-PR37.746.90.013Legend: * median (IQR)); ENT ear-nose-throat; GI gastrointestinal tract; PNS peripheral nervous system; CNS central nervous system;Conclusion:In our incipient AAV cohort 13% of patients presented with ILD. The AAV patients with ILD had a higher mortality rate than the rest of the cohort.References:Acknowledgments:Disclosure of Interests: :ALOJZIJA HOCEVAR: None declared, Katja Perdan-Pirkmajer: None declared, Matija Tomsic: None declared, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi.

Background:Previous studies have suggested similar effectiveness, but longer treatment retention, for tumor necrosis factor inhibitors (TNFi), when used in combination with a conventional synthetic disease modifying anti-rheumatic drug (csDMARD) in psoriatic arthritis (PsA).Objectives:To describe patients with PsA initiating a first TNFi as monotherapy compared to combination therapy, and to explore 1-year treatment retention of TNFi in the two groups.Methods:Patients with PsA starting a first TNFi (2006-2017) were identified in biologics registers of 13 European countries, and data were pooled for analysis. Co-medication with csDMARD was determined at TNFi start.Because of large inter-country variation in TNFi retention, countries were split into two strata, depending on each country’s 1-year retention rate for TNFi being above (stratum A) or below (stratum B) the average 1-year retention rate.TNFi treatment retention was compared through Kaplan-Meier curves; the proportion remaining on the TNFi at one year; and hazard ratios (HR) during the first year: (i) crude; adjusted for (ii) country-strata, and (iii) country-strata, sex, age, calendar year, DAS28 and disease duration. In model (iii) only registers contributing >1000 patients or Results:A total of 14778 patients with PsA starting a first TNFi were included. Baseline disease activity was similar within stratum B, but higher for the combination treatment group in stratum A (table 1).Table 1.Baseline characteristicsCountry strataStratum AStratum BTNFimonotherapyN=2120TNFi/csDMARDcombinationN=2128TNFimonotherapyN=3369TNFi/csDMARDcombinationN=7161Females52%51%53%51%Age, years49.7 (12.2)48.7 (11.8)48.8 (13.0)48.9 (12.2)Disease duration, yrs6.4 (7.0)6.8 (6.8)5.9 (7.5)5.9 (7.1)Tender joints 285.5 (6.3)8.0 (6.3)5.6 (6.0)5.6 (5.7)Swollen joints 282.8 (4.3)5.6 (5.0)3.0 (3.8)3.3 (3.8)VAS pain54 (29)62 (24)59 (23)56 (24)DAPSA-2824.6 (18.6)36.2 (17.6)27.3 (15.6)27.2 (15.2)DAS28 (CRP)3.5 (1.4)4.7 (1.3)4.0 (1.2)4.0 (1.1)Concomitant csDMARDMethotrexate-76%-79%Sulfasalazine-15%-15%Other csDMARD-49%-25%Numbers are means (sd) unless otherwise stated.The Kaplan-Meier curves for the treatment groups were similar within each stratum (fig 1), as were the proportions remaining on TNFi after one year, stratum A: monotherapy 86% (95%CI: 85-88) vs. combination 86% (84-87), stratum B: 71% (69-72) vs. 73% (72-74). The HRs for TNFi discontinuation (ref=TNFi monotherapy) were: (i) 1.06 (0.98-1.13), (ii) 0.94 (0.87-1.01), (iii) 0.89 (0.83-0.96), including 13078 patients (9 countries) for model (iii).Conclusion:In this exploratory study no benefit in TNFi retention was observed for csDMARD combination therapy in crude analyses, while in adjusted analyses an 11% lower risk of TNFi discontinuation was found. These preliminary results offer limited support for use of combination therapy in PsA. Further analyses will explore to what extent the results are affected by inter-country heterogeneity and differences between TNFi.Acknowledgments:UL and DDG contributed equally.Novartis Pharma AG and IQVIA support the EuroSpA collaboration.Disclosure of Interests:Ulf Lindström: None declared, Daniela Di Giuseppe: None declared, Bénédicte Delcoigne: None declared, Bente Glintborg Grant/research support from: Grants from Pfizer, Biogen and Abbvie, Burkhard Moeller: None declared, Manuel Pombo-Suarez Consultant of: Janssen, Lilly, MSD and Sanofi., Speakers bureau: Janssen, Lilly, MSD and Sanofi., Carlos Sánchez-Piedra: None declared, Kari Eklund Consultant of: Celgene, Lilly, Speakers bureau: Pfizer, Roche, Heikki Relas Grant/research support from: Abbvie., Consultant of: Abbvie, Celgene, and Pfizer., Speakers bureau: Abbvie, Celgene, and Pfizer., Björn Gudbjornsson Speakers bureau: Novartis and Amgen, Thorvardur Love: None declared, Gareth T. Jones Grant/research support from: Pfizer, AbbVie, UCB, Celgene and GSK., Adrian Ciurea Consultant of: Consulting and/or speaking fees from AbbVie, Bristol-Myers Squibb, Celgene, Eli Lilly, Merck Sharp & Dohme, Novartis and Pfizer., Catalin Codreanu Consultant of: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Speakers bureau: Speaker and consulting fees from AbbVie, Accord Healthcare, Alfasigma, Egis, Eli Lilly, Ewopharma, Genesis, Mylan, Novartis, Pfizer, Roche, Sandoz, UCB, Ruxandra Ionescu Consultant of: Consulting fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Speakers bureau: Consulting and speaker fees from Abbvie, Eli-Lilly, Novartis, Pfizer, Roche, Sandoz, Lucie Nekvindova: None declared, Jakub Zavada Speakers bureau: Abbvie, UCB, Sanofi, Elli-Lilly, Novartis, Zentiva, Accord, Nuh Atas: None declared, Servet Yolbaş: None declared, Karen Fagerli: None declared, Brigitte Michelsen Grant/research support from: Research support from Novartis, Consultant of: Consulting fees Novartis, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Matija Tomsic: None declared, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Maria Jose Santos Speakers bureau: Novartis and Pfizer, Pedro Ávila-Ribeiro Grant/research support from: Novartis, Lykke Midtbøll Ørnbjerg Grant/research support from: Novartis, Mikkel Ǿstergaard Grant/research support from: AbbVie, Bristol-Myers Squibb, Celgene, Merck, and Novartis, Consultant of: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo Nordisk, Orion, Pfizer, Regeneron, Roche, Sandoz, Sanofi, and UCB, Speakers bureau: AbbVie, Bristol-Myers Squibb, Boehringer Ingelheim, Celgene, Eli Lilly, Hospira, Janssen, Merck, Novartis, Novo Nordisk, Orion, Pfizer, Regeneron, Roche, Sandoz, Sanofi, and UCB, Lennart T.H. Jacobsson Consultant of: AbbVie, Eli Lilly, Janssen, Novartis and Pfizer, Johan Askling Grant/research support from: JA acts or has acted as PI for agreements between Karolinska Institutet and the following entities, mainly in the context of the ARTIS national safety monitoring programme of immunomodulators in rheumatology: Abbvie, BMS, Eli Lilly, Merck, MSD, Pfizer, Roche, Samsung Bioepis, Sanofi, and UCB Pharma, Michael Nissen Grant/research support from: Abbvie, Consultant of: Novartis, Lilly, Abbvie, Celgene and Pfizer, Speakers bureau: Novartis, Lilly, Abbvie, Celgene and Pfizer

Background:Objectives:To evaluate the frequency of cranial and aortic arch artery involvement in GCA using color Doppler ultrasonography (CDS).Methods:We performed CDS of cranial and aortic arch arteries in 248 incipient, clinically diagnosed, GCA patients (64.9% females, median (IQR) age 75 (67-80) years) between October 2013 and September 2019, using a Philips IU22 with 5–17.5 MHz linear probe or Philips Epiq 7 with 5–18.5 MHz linear probe. Temporal, facial, occipital, carotid, vertebral, subclavian, and axillary arteries were examined bilaterally. A halo with positive compression sign was considered a positive finding. Additionally, the thickness of intima-media complex (IMT) of individual vessel was measured, and compared to the IMT of 97 consecutive suspected GCA cases (60.8% females median (IQR) age 74 (65-81) years), in whom GCA was excluded, that served as a control group.Results:The CDS was positive in 244 (98.4%) patients in at least one of the examined arteries. Temporal arteries were most commonly affected, and were involved in 192 (77.4%) patients, followed by facial and occipital arteries, involved in 122 (49.2) and 72 (29.0%) patients, respectively. Extracranial large vessel involvement (LVV) was found in 87 (35.1%) patients (32 patients had isolated LVV, and 55 concomitant cranial and LVV artery involvement). Among the 161 patients without LVV, 12 (4.8% of the studied cohort) had involvement of cranial arteries other than temporal arteries (we found facial and occipital artery involvement in 11 and 3 patients, respectively). Table 1 shows the frequency of individual vessel involvement in GCA, and the IMT of CDS inflamed and non-inflamed arteries in GCA, and in controls.Table 1.The involvement of cranial and aortic arch arteries in GCA assessed by CDS and intima-media thickness of inflamed and non-inflamed arteries in GCA, and controlsArteryGCA No (%)IMT (mm) in GCAIMT (mm) in ControlsPositive CDSPositive CDS*; minimalNegative CDSNegative CDS; maximalTemporal192 (77.4)0.71±0.19; 0.330.25±0.070.23±0.05; 0.46Facial122 (49.2)0.75±0.27; 0.410.29±0.070.26±0.07; 0.47Occipital72 (29.0)0.73±0.33; 0.450.26±0.060.23±0.05; 0.46Carotid34 (13.7)1.53±0.44; 0.880.78±0.180.72±0.15; 1.09Vertebral25 (10.1)1.33±0.47; 0.740.45±0.100.42±0.08; 0.63Subclavian67 (27.0)1.65±0.45; 0.910.70±0.140.70±0.13; 0.99Axillary59 (23.8)1.74±0.65; 1.000.61±0.170.57±0.13; 0.97Any artery244 (98.4)---Legend: GCA giant cell arteritis; IMT thickness of intima-media complex; * mean±SD;Conclusion:CDS of seven preselected cranial and aortic arch arteries provides a high diagnostic yield in GCA.Disclosure of Interests:Rok Jese: None declared, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Matija Tomsic: None declared, ALOJZIJA HOCEVAR: None declared

Due to the well-established risk of latent tuberculosis infection (LTBI) reactivation, and its potentially devastating consequences in patients treated with tumour necrosis factor (TNF)-α inhibitors (anti-TNF), a low threshold for TB chemoprophylaxis is usually recommended.1 ,2 Conversely, TB chemoprophylaxis is time consuming, delays the initiation of required treatment, adds to the overall cost of treatment, and carries risk of adverse events in its own right.3 A screening algorithm able to safely reduce the frequency of TB chemoprophylaxis would be of great value. Recently, Mariette et al 4 showed that replacing the tuberculin skin test (TST) with interferon-γ release assays in their LTBI screening algorithm reduced the requirement for TB chemoprophylaxis from 45.2% to 27.3%, while there were no new cases of TB during the 1-year observation period. In Slovenia, since 2002, a two-step LTBI screening algorithm is being used for all rheumatological patients who are candidates for anti-TNF. The LTBI screening is not repeated on a regular …

The 2017 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for idiopathic inflammatory myopathies (IIM) are the first validated classification criteria for IIM. The criteria provide a score with a corresponding probability of having IIM. The cut-offs for classifying a patient with IIM were set at ≥5.5 and ≥6.7 in the probability models without and with available muscle biopsy results, respectively. At these cut-offs, the criteria had an 87% and 93% sensitivity, and 82% and 88% specificity, in the model without and with the muscle biopsy results. The criteria were tested for sensitivity, but not for specificity in two independent cohorts.1 We evaluated the performance …

Background Polyarteritis nodosa (PAN) is a necrotising vasculitis of medium and small sized muscular arteries. It is a rare disease, with reported annual incidence rate of 1–8 cases per 10 6 adults. 1,2 In areas where hepatitis B virus (HBV) infection is hyperendemic however, the annual incidence rate is much higher, i.e. up to 77 per 10 6 adults. 3 Objectives To determine the annual incidence rate of PAN within a well-defined adult population of the Ljubljana region of Slovenia. Methods A retrospective chart review of adult patients diagnosed with PAN was performed at the Department of Rheumatology of University Medical Centre Ljubljana, which is a part of an integrated secondary/tertiary university teaching hospital, that is the only referral rheumatology centre in the Ljubljana region, which represents about a quarter of Slovenian population. Potential cases were identified by searching the electronic medical records for ICD-10 codes M30.0. The electronic and paper records of patients who were residents of the Ljubljana region at the time of diagnosis were then thoroughly reviewed. Cases fulfilling the ACR 1990 classification criteria for PAN with either histologically proven vasculitis or characteristic arteriographic abnormalities were included in the analysis. The annual incidence rate of PAN was then calculated. Results Between January 1, 2003 and December 31, 2012 (10 years of observation) five new cases (3 male, 2 female, male:female ratio=1,5; median age 49 (range 27–72) years) of PAN were identified in the Ljubljana region with a population of 527,000 aged 18 years or above. Patients9 clinical characteristics at the time of diagnosis are presented in Table 1. Four patients complained of myalgias and all male patients had testicular pain. Peripheral nerve lesions were identified in two. Three patients had arteriographic abnormalities; two of them had the characteristic microaneurysms. One patient had an active HBV infection. One had a localized cutaneous form of PAN on the basis of nodular skin changes with typical histological picture and no other organ system involvement (Case 1*). During the follow up one patient developed AA amyloidosis. Conclusions The estimated annual incidence rate of PAN was 0.95 per 10 6 adults. References Gonzalez-Gay, MA, et al. Arthrit Care Res, 2003, 49.3: 388-93. Watts, RA, et al. Arthritis Rheum, 2000, 43.2: 414-19. McMahon, BJ, et al. Hepatology, 1989, 9.1: 97-101. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2564