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INTRODUCTION Behcet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described. OBJECTIVES To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort. METHODS A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score >4 points. RESULTS We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N=243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR=2.004, p=0.024), increasing with age (OR=1.021 per year, p=0.037), in the presence of skin manifestations (OR=4.711, p

Background Irreversible organ damage is considered a core outcome by the OMERACT working group. However, no specific tools are currently available to detect and measure damage accrual in Behcet’s syndrome (BS). Objectives To develop and preliminarily validate the Behcet’s syndrome Overall Damage Index (BODI). NCT03803462. Methods A preliminary version of the instrument (p-BODI) was developed by reviewing pre-existing tools [e.g. Vasculitis damage index (VDI)] and through an extensive literature review. p-BODI was then reviewed and implemented by a multi-rounds Delphi process, involving an international and multidisciplinary (5 rheumatologists, 4 internist, 1 ophthalmologist, 1 neurologist) panel of experts in BS management and a patients’ delegate. A group of clinicians (CG), not involved in the BODI development, was asked to independently score a set of clinical vignettes, in order to test the instrument reliability, after a training process consisting of a user manual and a video-tutorial. Then, Cohen’s K and Intra-class correlation coefficient (ICC) between assessors and gold standard were calculated. Afterwards, BODI validation was conducted according to the OMERACT Filter 2.0 in a multicenter BS cohort. Results Starting from a list of 120 candidate items, the final version of BODI consisted of 4 overarching principles, 30 items and 12 sub-items (each of them scores one point) grouped in 8 domains (figure). In terms of reliability, the mean K coefficient was 0.84 (95%CI 0.78 to 0.90) and the ICC was 0.88 (95%CI 0.80-0.95). Validation cohort consisted of 228 BS patients (49.1% males), with a median (IQR) age and disease duration of 46.9 (35.5-55.0) and 11.7 (5.8-20.7) years, respectively. Overall, prevalence of any BODI damage (BODI ≥1) was 56.1% with a median score of 1.0 (0-2.0). In regard of construct validity, BODI score significantly correlated with VDI (Spearman’s rho 0.693, p Full agreement among the CG was reached in judging BODI as a credible, comprehensive, easy to use, timesaving and acceptable instrument. Conclusion BODI is the first tool specifically developed to assess damage in BS. Preliminary data encourage further validation of BODI in more extended and multi-ethnic BS cohorts before being applied in clinical practice and as a therapeutic outcome. Disclosure of Interests Matteo Piga: None declared, Alberto Floris: None declared, Gerard Espinosa: None declared, Nikolaos Kougkas: None declared, Andrea Lo Monaco: None declared, Giuseppe Lopalco Speakers bureau: SOBI, BMS, Ida Orlando: None declared, Vittorio Pirani: None declared, Ernestina Santos: None declared, Luisa Serpa Pinto: None declared, George Bertsias: None declared, Luca Cantarini: None declared, Alberto Cauli: None declared, Ricard Cervera: None declared, Joao Correia: None declared, Marcello Govoni: None declared, Florenzo Iannone Consultant for: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Speakers bureau: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Ana Martins da Silva: None declared, Piergiorgio Neri: None declared, Carlos Vasconcelos: None declared, Monica Muntoni: None declared, Alessandro Mathieu: None declared

Background Disease activity evaluation is mandatory according to the OMERACT Core Set of Domains for Behcet’s Syndrome (BS). Poor data are available on Patient (PtGA) and physician (PGA) global assessment of disease activity in BS. Objectives To assess PtGA performance in patients with BS and how different disease manifestations influence the patient and physician’s perception of disease activity. Methods A multicenter cross-sectional cohort of consecutive BS patients was enrolled. Disease activity was evaluated by PtGA, PGA and the Behcet’s disease current activity form (BDCAF). PtGA and PGA were assessed trough a single question (“How active was BS during the last week?”) in a 10-cm visual analogic scale. Health related quality of life (HRQoL) perception was evaluated by the Physical Component Summary (PCS) and the Mental Component summary (MCS) of the SF36v2 questionnaire. Correlation between PtGA, PGA, BDCAF and between them and SF36v2 component summaries was assessed trough the Spearman’s correlation coefficient (rho). Two separated multiple regression models were built to measure the independent effect (β regression) of each active clinical manifestation on PtGA and PGA. Results Overall, 226 BS patients from 5 Mediterranean Countries were enrolled. Out of them, 111 (49.1%) were males and the median (IQR) age and disease duration were 46.9 (35.6-55.2) and 11.7 (5.9-20.8) years, respectively. The median (IQR) value of PtGA, PGA and BDCAF were 2.0 (0.3-5.0), 1.0 (0.0-3.0) and 3.0 (0.0-5.0), respectively. PtGA significantly correlated (Figure) with both PGA (rho 0.759. p 0.001) and MCS (rho -451. p > 0.001). Conclusion PtGA seems to be a valid outcome measure in BS and should be considered as a separate outcome measure or better be included in composite index for overall disease activity evaluation. Disclosure of Interests Alberto Floris: None declared, Matteo Piga: None declared, Gerard Espinosa: None declared, Nikolaos Kougkas: None declared, Andrea Lo Monaco: None declared, Giuseppe Lopalco Speakers bureau: SOBI, BMS, Ida Orlando: None declared, Vittorio Pirani: None declared, Ernestina Santos: None declared, Luisa Serpa Pinto: None declared, George Bertsias: None declared, Luca Cantarini: None declared, Alberto Cauli: None declared, Ricard Cervera: None declared, Joao Correia: None declared, Marcello Govoni: None declared, Florenzo Iannone Consultant for: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Speakers bureau: F Iannone has received consultancy fees and/or speaker honoraria from Pfizer, AbbVie, MSD, BMS, Novartis, Lilly, UCB outside this work, Ana Martins da Silva: None declared, Piergiorgio Neri: None declared, Carlos Vasconcelos: None declared, Alessandro Mathieu: None declared

A 73-year-old woman without a history of cardiovascular disease was admitted to the hospital with progressive dyspnoea on exertion during the past several weeks, bilateral lower extremity oedema, orthopnoea and paroxistic nocturnal dyspnoea. She lived a few metres away from a badly preserved building containing asbestos for 50 years. Estimated jugular venous pressure was 6+5 cm H2O, a pericardial friction rub was heard, bilateral basal lung crackles were present and symmetrical below-knee pitting oedema was seen. Probrain natriuretic peptide was 2304 pg/mL, and chest radiograph showed a globular cardiac silhouette and blunting …