Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared

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Background:Schnitzler’s syndrome is an autoinflammatory disease characterized by monoclonal gammopathy and recurrent episodes of urticaria accompanied by clinical and laboratory signs of acute inflammation. Interleukin (IL)-1 inhibitors proved to be useful in the treatment, but data on long-term safety and efficacy of these agents are sparse.Objectives:To evaluate the retention rate of IL-1 inhibitors in patients with Schnitzler’s Syndrome.Methods:Retrospective analysis of an Italian multicenter cohort (9 Centers). All patients fulfilled Strasbourg diagnostic criteria. Data are expressed as median [IQR].Results:We identified 15 patients (8 females, 7 males) who received a total of 24 treatment courses with IL-1 inhibitor treatment (16 anakinra and 8 canakinumab) between January 2001 and December 2019, with a median treatment duration of 19 months [8.5-51.3]. Median age at diagnosis was 64.0 years [56.0-72.5] and median follow up was 5.0 years [2.0-8.0]. Before the biological treatment, all patients were treated with corticosteroids and 11 with at least one conventional synthetic disease-modifying antirheumatic drug (csDMARD): methotrexate (5), colchicine (5), cyclosporine (3), azathioprine (1), mycophenolate mofetil (1), cyclophosphamide (1).Fifteen patients received 16 courses of Anakinra, which was the 1st line biological treatment in 14 patients. Seven patients continued it with benefit, while 7 patients discontinued it: 3 for secondary inefficacy; 3 for adverse events (2 injection site reactions, 1 severe allergic reaction); 1 for secondary inefficacy and leukopenia. Anakinra was used as 2nd line treatment in 1 case (after tocilizumab failure); in 1 patient anakinra was resumed after temporary discontinuation and an attempt with infliximab. One patient died for multiple myeloma progression while on treatment with anakinra. The median duration of the courses with anakinra was 20.0 months [6.0-58.3].Seven patients received 8 courses of canakinumab (150 mg/8weeks in 5 cases and 150 mg/4weeks in 3). In 5 cases the drug was administered as 2nd line biological treatment (after anakinra failure) and in 2 cases as 3rd line treatment (1 after tocilizumab and anakinra failures and 1 after anakinra and adalimumab failure). In 1 patient, it was resumed after temporary discontinuation and an attempt with etanercept. One patient died while on treatment with canakinumab due to a presumably unrelated adverse event. The median duration of canakinumab treatment courses was 19.0 months [13.5-31.0].At last follow-up visit, all patients were on treatment with an IL-1 inhibitor: 8 with anakinra (7 at the dosage of 100 mg/day, 1 at the dosage of 200 mg/day) and 7 with canakinumab (2 at the dosage of 150 mg/8 weeks, 4 at the dosage of 150 mg/4 weeks and 1 at the dosage of 300 mg/4 weeks). Notably, in 3 patients the dosage of canakinumab was increased since the start of the treatment.Among 9 patients who were on treatment with prednisone at the start of the last IL-1 inhibitor, the prednisone median dose was 12.5 mg/day [10.0-18.8] while at the last follow-up visit it was 5.0 mg/day [0-7.5] (p= 0.02).The retention rate of IL-1 inhibitors was 73.4% [SE 9.4] at 1 year and 63.6% [SE 10.4] at 2 years (Figure 1a). There was no significant difference between the retention rate of anakinra (at 1 year: 67.0% [12.2]; at 2 years: 59.6% [12.9]) and canakinumab (at 1 year: 85.7% [13.2]; at 2 years 71.4% [17.1]) (log-rank test: p=0.41) (Figure 1b).Figure 1.a) Retention rate of IL-1 inhibitors (24 courses); b) Retention rate of canakinumab (8 courses) and anakinra (16 courses).Conclusion:In this multicentric cohort of patients affected by Schnitzler’s syndrome, the treatment with IL-1 inhibitors as 1st, 2nd or 3rd line biological treatment permitted a good disease control and corticosteroid reduction in patients who did not respond to csDMARDs and/or to prior other biological DMARDs. The optimal dosage of these drugs needs to be tailored for every patient.Acknowledgements:AIDA NetworkDisclosure of Interests:Francesca Crisafulli: None declared, Antonio Vitale: None declared, Carla Gaggiano: None declared, Lorenzo Dagna: None declared, Giulio Cavalli Speakers bureau: SOBI, Novartis, Paid instructor for: SOBI, Novartis, Consultant of: SOBI, Novartis, Rolando Cimaz: None declared, Ombretta Viapiana: None declared, Florenzo Iannone: None declared, Giuseppe Lopalco: None declared, Roberto Bortolotti: None declared, Masen Abdel Jaber: None declared, Carlomaurizio Montecucco: None declared, Sara Monti: None declared, Silvia Balduzzi: None declared, Giacomo Emmi: None declared, Paolo Airò: None declared, Franco Franceschini: None declared, Luca Cantarini Speakers bureau: SOBI, Novartis, Paid instructor for: SOBI, Grant/research support from: SOBI, Novartis, Micol Frassi: None declared

Background The performance of the 2010 ACR/EULAR classification criteria for rheumatoid arthritis (RA) using ultrasonography (US) as an alternative instrument to detect synovitis has recently been tested (1). Objectives To evaluate the impact of the addition of US on the performance of the 2010 criteria in early arthritis patients, considering the use of disease modifying antirheumatic drugs (DMARDs) 12 months after the first evaluation as reference standard for diagnosis. Methods Patients attending for the first time an early arthritis clinic before the 2010 criteria were published, presenting with at least one swollen joint and no alternative diagnosis, were included. US of bilateral wrists and metacarpophlangeal joints (1-5) was performed at baseline; Grey-scale (GS) and power-Doppler (PD) synovitis were semi-quantitatively scored(0-3). Overall GS and PD scores were obtained as the sum of the score of each joint. RA patients (1987 criteria) were given methotrexate (MTX) or, if contraindicated, other DMARDs (sulphasalazine, leflunomide, cyclosporine), undifferentiated polyarthritis patients received hydroxichloroquine(HCQ). Patients were seen every two months in the first semester and then every three. HCQ was substituted by MTX, MTX escalated up to the maximum tolerated dose and biologics added if Disease Activity Score was ≥2.4 at subsequent visits. The 2010 classification criteria were applied at baseline, the use of synthetic or biologic DMARDs (except HCQ) after 12 months was considered as reference standard. Logistic regression was applied to evaluate the predictive value of GS and PD scores. The additional value of GS score and PD scores on the performance of the criteria was tested using the likelihood ratio test. The performance of the criteria and of the addition of US was evaluated examining the area under the curve (AUC). Only patients with complete clinical and US data were included. Results 188 patients with complete data, from a larger cohort, were included, 74% were female, mean age (sd) was 57.6 (±15.0), median (IQR) disease duration 3.07 (1.2,7.1) months, 32% were rheumatoid factor positive, 69.1% had RA according to the 1987 criteria, 80.8% according to the 2010 criteria. After 12 months 157/188 (83.5%) patients were taking DMARDs. Using 12 month DMARDs as reference standard, the AUC for the 2010 classification criteria was 0.82. GS score predicted DMARD use with an odds ratio (OR) (95% CI) of 1.14 (1.04,1.25). The addition of GS scores ≥5 but <10 led to an OR of 1.25 (0.51,3.02), while scores ≥10 had an OR of 5.13 (1.62,16.18). The addition of the GS score to the criteria increased the AUC to 0.85 (p=0.05). PD score predicted DMARD use as well (OR 1.15 (1.02,1.29)). PD scores ≥2 led to an OR of 2.74 (1.0,7.52), and the addition of PD score (cut-off of 2) to the criteria significantly improved their performance (AUC 0.85, p<0.05). Conclusions The addition of single US features (GS and PD scores) significantly improves the performance of the 2010 RA classification criteria. The integr...