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Interstitial lung disease (ILD) has been described as a possible pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), mainly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Aim of this cross-sectional Italian national study was to describe demographic, clinical and serological profile of ILD related to MPA and GPA and investigate possible correlations between radiologic patterns of ILD and vasculitis features.We enrolled 95 consecutive patients with AAV-ILD, 56 affected by MPA (58.9%) and 39 by GPA (41.1%).NSIP was the most frequently detected ILD pattern, observed in c-ANCA patients in 60.9% of cases, followed by UIP pattern mainly observed in p-ANCA patients (47.7%, p=0.03). ILD represented the first clinical manifestation, preceding vasculitis diagnosis in 22.1% of cases and, globally, ILD was already detectable at AAV diagnosis in 66.3% of patients. The diagnosis of ILD preceded that of AAV in 85.7% of p-ANCA positive-patients, while only one patient with c-ANCA developed ILD before AAV (p= 0.039). Multivariate analysis confirmed the correlation of UIP pattern with p-ANCA-positivity and a diagnosis of ILD before AAV, also when adjusted for age and sex.Our study confirms that UIP is a frequent pattern of lung disease in AAVILD patients. Our results also suggest that ILD can represent an early complication of AAV but also occur in the course of the disease, suggesting the need of a careful evaluation by both pulmonologist and rheumatologist to achieve an early diagnosis. Further prospective studies are needed to define ILD prevalence and evolution in AAV patients.

The provisional EULAR recommendations address several aspects of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus, and the disease caused by SARS-CoV-2, COVID-19 and are meant for patients with rheumatic and musculoskeletal diseases (RMD) and their caregivers. A task force of 20 members was convened by EULAR that met several times by videoconferencing in April 2020. The task force finally agreed on five overarching principles and 13 recommendations covering four generic themes: (1) General measures and prevention of SARS-CoV-2 infection. (2) The management of RMD when local measures of social distancing are in effect. (3) The management of COVID-19 in the context of RMD. (4) The prevention of infections other than SARS-CoV-2. EULAR considers this set of recommendations as a 'living document' and a starting point, which will be updated as soon as promising new developments with potential impact on the care of patients with RMD become available.

Background and objectiveSystemic small vessel vasculitides carry an increased risk of acute arterial and venous thromboembolic events (AVTE); however, this risk has not been systematically explored in Eosinophilic Granulomatosis with Polyangiitis (EGPA). This study assessed the occurrence and main risk factors of AVTE among EGPA patients as compared to the general community from the population-based Bruneck cohort.MethodsWe conducted a retrospective multicenter cohort study on 573 EGPA patients. Clinical and serological data were collected at diagnosis. Occurrence of AVTE and time to the first AVTE after EGPA diagnosis were recorded. Age-standardized event rate (SER) of AVTE as compared to the reference cohort was assessed. Cox regression was applied to identify AVTE predictors.Results129 EGPA patients (22.5%) had AVTE, considered as potentially life-threatening in 55.8%. Seventy patients experienced an AVTE prior to diagnosis (of whom 58.6% in the two years before diagnosis) and 75 following EGPA diagnosis, of whom 56% in the two subsequent years. The SER of AVTE as compared to the reference cohort was 2.10 (95% CI 1.67-2.63). This risk was particularly increased in patients with history of AVTE and with a Birmingham Vasculitis Activity Score ≥20 at diagnosis. Patients receiving immunosuppression within 2 months of diagnosis were at lower risk, while antiplatelet or anticoagulant treatment did not confer measurable benefit.ConclusionEGPA is associated with AVTE in approximately one quarter of patients, particularly around diagnosis. Immunosuppressants seemed to exert a protective effect, while anticoagulant and antiplatelet agents did not.

Introduction Alveolar haemorrhage (AH) is considered an important cause of morbidity and early mortality in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV). Objectives The aim of this study was to identify predictors of outcome in patients with AH-AAV and to evaluate outcome and causes of death in this subset. Materials and methods A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed. Results One-hundred and six patients were included (median age at onset of 55 years [IQR 42–67]). The majority were ANCA-positive (PR3 57.1%, MPO 33.7%) and 72.6% had also renal involvement. At presentation, anaemia was shown in 97 (92.4%) patients, hemoptysis in 54 (51.9%), respiratory failure in 68 (66.7%), of whom 48 (70.6%), requiring respiratory support. At the end of the 37 months [IQR 13–77] follow-up, 19/106 (17.9%) patients were dead. The main causes of death were active disease and infections. By stepwise regression analysis, age >65 years (HR 3.66 [95% CI 1.4–9.51], p = 0.008) and the need for respiratory support (HR 4.58 [95% CI 1.51–13.87], p = 0.007) at AH onset were confirmed to be predictive of mortality. Conclusions Predictors of outcome in AAV-AH were determined. Factors related to the patient's performance status and the severity of the lung involvement strongly influenced the outcome. Balancing harms and benefits for the individual patient in induction and maintenance treatment strategies is crucial.

Objectives Recent data have shown a significant efficacy of rituximab (RTX) in SSc. An RTX biosimilar (RTX-B) is a more affordable option. We assessed the safety and efficacy of an RTX-B (CT-P10) in SSc. Methods SSc patients treated with RTX-B with at least 6 months of follow-up were retrospectively identified from six Italian referral centres. SSc patients naïve to RTX-B (RTX-Bn) or already treated with RTX originator and switched to an RTX-B (RTX-Bs) were evaluated. A comprehensive assessment of disease characteristics and organ involvement at baseline and after 6 months was obtained. Results Thirty-three SSc patients were selected: 29 (87.9%) females, mean age 51.6 years (s.d. 14.2), mean disease duration 9.8 years (s.d. 8.1); 21 (64.5%) with dcSSc, 20 (60.1%) anti-topoisomerase I, 7 (21.2%) anti-RNA polymerase III and 6 (18.2%) anti-centromere positive. Seventeen (51.5%) were RTX-Bn and 16 were on RTX-Bs (48.5%). RTX was introduced because of skin progression in 18 patients (54.5%), interstitial lung disease (ILD) worsening in 11 (33.3%) and arthritis in 12 (36.4%). All patients were previously treated with immunosuppressants. At RTX-B introduction, 21 (63.6%) patients were on concomitant immunosuppressants: 15 (71.4%) on MMF and 6 (28.6%) on MTX. Twenty-three (69.7%) were on low-dose steroids. After 6 months, a significant reduction of the modified Rodnan skin score (mRSS), 28-joint DAS and CRP was observed (P = 0.002, 0.005 and 0.008, respectively); the mRSS significantly improved both in RTX-Bn (P Conclusion Our data suggest that RTX-B can represent a cheaper option in SSc patients, as it is effective in improving skin and joint involvement and in stabilizing lung function.

Background:Rituximab (RTX), an anti-CD20 monoclonal antibody, represents a valuable treatment for anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). RTX biosimilar CT-P10 (RTX-B) has been approved in Europe in all indications held by RTX originator (RTX-O). As stated by recent international consensus-based recommendations, 1 there is evidence regarding safety and efficacy of biosimilars in the context of rheumatic diseases, but it is encouraged to gather additional data. 2,3Objectives:To report the experience of a single Italian center with RTX-B in AAV in terms of safety and efficacy.Methods:We retrospectively reviewed the charts of all AAV patients followed up in our Small Vessel Vasculitis Clinic and we selected those who received RTX-B between October 2017 and May 2020, both naïve to RTX (RTX-Bn) or already treated with ≥1 course of RTX-O and switched to RTX-B (RTX-Bs). Baseline features, disease outcome, concomitant therapy and adverse events 6 (T6), 12 (T12) and 24 (T24) months after RTX-B introduction, when available, were collected. Non-parametric statistic tests were used.Results:Fifty-six AAV patients (44 [78.6%] granulomatosis with polyangiitis (GPA), 12 [21.4%)] microscopic polyangiitis (MPA)) received RTX-B with a median follow up of 20 (IQR 10-24) months. ANCA were positive in 49 (87.5%) patients. Ten (17.9%) patients were newly diagnosed with AAV, while 23 (41.1%) had refractory disease and another 23 had relapsing disease.Thirty-three (58.9%) patients were RTX-Bs, whereas 23 (41.1%) RTX-Bn. In 29 (51.8%) patients RTX was decided because of remission induction, while in 27 (48.2%) as maintenance regimen. Median cumulative RTX-B dose was 2.5 (2-3.875) grams with 3 (IQR 2-4) median courses. AAV activity, adverse events and concomitant therapy at T6, T12 and T24 are shown in Table 1. One GPA patient died of severe infection 3 months after a single infusion of RTX-B 500 mg as maintenance therapy.Table 1.AAV activity, concomitant therapy and adverse events at T0, T6, T12 and T24.T0T6T12T24n (%)56 (100)52 (92.6)40 (71.4)18 (32.1)Dose RTX-B, median (IQR) grams1.75 (0.5-2)0.5 (0.5-1)0.5 (0.5-1)0.5 (0.5-1)Concomitant prednisone therapy, n (%)42 (75)27 (48.2)13 (32.5)4 (22.2)Dose prednisone, median (IQR) milligrams/day5 (5-15.625)2.5 (2.5-5)3.75 (2.75-7.5)2.75 (1.56-4.5)Concomitant bDMARDs, n (%)10 (17.9)5 (9.6)3 (7.5)1 (5.6)RefractorinessNA4 (7.7)2 (5)1 (5.6)RelapseNA0 (0)3 (7.5)2 (11.1)RemissionNA48 (92.3)35 (87.5)15 (83.3)SuspensionNA10 (19.2)9 (22.5)4 (22.2)Infusion reaction, n (%)1 (1.8)1 (1.9)0 (0)0 (0)Infectious adverse events, n (%)NA10 (19.2)6 (15)3 (16.7)Severe infectious adverse events, n (%)NA2 (3.8)1 (2.5)0 (0)Considering the 52 (92.6%) patients who reached a 6 months-follow up, we marked a significant decrease of number of patients on steroid therapy (39 [75%] vs 27 [51.9%]; p=0.003), of median prednisone daily dose (5 [IQR 5-25] vs 2.5 [IQR 1.25-5 mg; p=0.001) and of number of patient on concomitant bDMARDs (10 [19.2%] vs 5 [9.6%]; p=0.003).Analysing the 40 (71.4%) patients who reached a 12 months-follow up, we confirmed the tendency with a decrease of number of patients on steroid therapy (29 [72.5%] vs 13 [32.5%]; p=0.007), of median prednisone daily dose (5 [IQR 5-12.5] vs 3.75 [IQR 2.75-7.5]; p=0.008) and of number of patient on concomitant bDMARDs (9 [22.5%] vs 3 [7.5%]; p=0.009).No statistically significant difference was found for the same variables comparing T0 versus T24.Conclusion:Our experience with RTX-B is limited; however, it represents an extensive report about the use of RTX-B in AAV patients and confirmed that it may be a well-tolerated and effective therapy in AAV setting, in agreement with previous evidence on RTX-O. Prospective and larger studies are needed to confirm this preliminary evidence.References:[1]Kay J, et al. Ann Rheum Dis 2018;77:165–74.[2]Kwon HC, et al. Yonsei Med J 2020;61:712-9.[3]Mittal S, et al. Clin Rheumatol 2021;40:645-51.Disclosure of Interests:Silvia Sartorelli: None declared, adriana cariddi: None declared, Luca Moroni: None declared, Elena Baldissera Speakers bureau: Pfizer, Roche, Sanofi-Genzyme, Consultant of: Novartis, Enrica Bozzolo: None declared, Lorenzo Dagna Consultant of: Abbvie, Amgen, Biogen, BristolMyers Squibb, Celltrion, Galapagos, GlaxoSmithKline, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unrestricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, GlaxoSmithKline, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

Background recent data support the use of rituximab(RTX) in Systemic Sclerosis(SSc). RTX biosimilar(RTX-B) offers a more affordable option but its efficacy and safety have not yet been evaluated. Objectives To assess the safety and efficacy of RTX-B in SSc. Methods Data about SSc patients treated with RTX-B(1gr repeated after 2 weeks) and with a follow-up ≥6 months were retrospectively collected from 5 Italian centres. Both SSc patients naive to RTX(RTX-Bn) or already treated with ≥1 course of RTX originator(RTX-O) and switched to RTX-B(RTX-Bs) were considered. A comprehensive assessment of disease features and organ involvement was available at baseline and at final follow-up for all patients. Non parametric tests were used. Results Data of 21 SSc patients(20 female, mean age 50.5±11.8 years) were collected;mean disease duration at RTX-B therapy was 7.6±4.8 yrs. Eleven patients(52%) had diffuse cutaneous SSc(dcSSc), 12(57%) were anti-topoisomeraseI+, 5 anti-RNA-polymeraseIII+ and 4 anti-centromere+. Twelve patients(57%) were RTX-Bn and 9 RTX-Bs(43%). In RTX-Bs patients, the median number of previous RTX-O courses was 3(range 1 – 8). RTX was decided because of skin progression in 11(52%), interstitial lung disease(ILD) worsening in 9(43%), arthritis in 6(29%), myocarditis and myositis in 1 patient each. All patients had been previously treated with immunosuppressants: mycophenolate mofetil(MMF) 14(67%), methotrexate(MTX) 7(33%), cyclophosphamide 6(29%), azathioprine 4(19%), tocilizumab and etanercept 1(5%) patient each. At RTX-B introduction, 14(67%) patients were on concomitant immunosuppressant: 10(48%) MMF and 4(19%) MTX; 15 patients(71%) were also on steroids(mean dose:3.1±2.1mg/day). At 6 months after RTX-B treatment, a significant reduction of the modified Rodnan skin score(mRSS), DAS28 and erythrocyte sedimentation rate(ESR) was observed in the entire cohort (p Conclusion: in agreement with previous data published on RTX-O, also RTX-B seem efficient in improving skin and joint involvement and in stabilizing lung function, either in RTX-Bn and in RTX-Bs SSc patients. Reference: [1] JordanS,et al.AnnRheumDis.2015;74(6):1188-94. Disclosure of Interests Corrado Campochiaro Consultant for: Dr Corrado Campochiaro received consultation honoraria from Pfizer., Giacomo De Luca Consultant for: Dr Giacomo De Luca received consultation honoraria from Pfizer and SOBI., Maria Grazia Lazzaroni: None declared, Silvia Laura Bosello: None declared, Maria De Santis: None declared, adriana cariddi: None declared, Carlo Selmi Grant/research support from: AbbVie, Janssen, MSD, Novartis, Pfizer, Consultant for: AbbVie, Alfa-Sigma, Biogen, Bristol-Myrs Squibb, Celgene, Eli-Lilly, Janssen, Merck Sharp and Dohme, Novartis, Pfizer, Roche, Sanofi-Genzyme, UCB, Speakers bureau: AbbVie, Alfa-Sigma, Biogen, Bristol-Myrs Squibb, Celgene, Eli-Lilly, Janssen, Merck Sharp and Dohme, Novartis, Pfizer, Roche, Sanofi-Genzyme, UCB, Elisa Gremese Consultant for: AbbVie, BMS, Celgene, Janssen, Lilly, MSD, Novartis, Sanofi, UCB, Roche, and Pfizer, Speakers bureau: BMS, Speakers bureau: Roche, Speakers bureau: AbbVie, BMS, Celgene, Janssen, Lilly, MSD, Novartis, Sanofi, UCB, Roche, and Pfizer, Paolo Airo: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Pfizer, BMS, Chemomab, Sanipedia, Speakers bureau: Actelion, BMS; MSD, Janssen, Lorenzo Dagna Consultant for: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Sanofi-Genzyme, and SOBI.

Background Giant cell arteritis (GCA) is a large-vessel vasculitis that generally affects people older than 50 years1. Glucocorticoids are the mainstay treatment2. Tocilizumab (TCZ) has recently been shown in clinical trials to be effective and safe for the treatment of GCA3. Objectives To describe our real-life experience with TCZ in a cohort of GCA patients from a single Italian Centre. Methods We included GCA patients diagnosed according to the 1990 aCR criteria, followed-up at our Large Vessel Vasculitis Clinic, and treated with TCZ. We retrospectively evaluated demographic features, disease characteristics, reasons for TCZ introduction and discontinuation, concomitant therapies and response to treatment. For each patient we evaluated potential eligibility for GiACTA trial. For statistical comparisons, we used non-parametric tests. Results 40 patients (32 women, mean age 71±10 years) were included. Table 1 summarizes clinical features, tools for GCA diagnosis (imaging and/or biopsy) and reasons for TCZ introduction. Mean disease duration before TCZ introduction was 21.5±29.1 months. Only 12 patients (30%) met both GiACTA inclusion and exclusion criteria at TCZ introduction. 31 patients were followed-up for 3 months, 23 for 6 months, 17 for 12 months. Mean prednisone (PDN) dose at TCZ introduction was 21.9±13.2 mg. Mean PDN dose was 8.4±6.5 mg (p Conclusion Our data confirm TCZ is highly effective in GCA treatment and has a significant steroid-sparing effect. In our real-life retrospective study, TCZ was also shown to be a safe option in patients with relapsing or refractory GCA. References [1] Salvarani C., et al. Polymyalgia rheumatica and giant-cell arteritis. Lancet (2008) [2] Proven a., et al. Glucocorticoid therapy in giant cell arteritis. Arthritis Rheum (2003) [3] Stone JH, et al. Trial of Tocilizumab in Giant-Cell arteritis. N Engl J Med (2017) Disclosure of interests Alessandro Tomelleri: None declared, Corrado Campochiaro Consultant for: Dr Corrado Campochiaro received consultation honoraria from Pfizer., Silvia Sartorelli: None declared, adriana cariddi: None declared, Elena Baldissera Consultant for: Consultation honoraria from Novartis and Rottapharm, Speakers bureau: Pfeizer, Sobi, Novartis, Lorenzo Dagna Consultant for: Prof Lorenzo Dagna received consultation honoraria from abbvie, amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Sanofi-Genzyme, and SOBI.

Background:Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a systemic disease characterized by late onset asthma associated with small- and/or medium-size vessel vasculitis, besides eosinophil-mediated cytotoxic organ damage. About 20-30% of patients with EGPA displays allergic manifestations related with inhalant sensitization, while prevalence of food and drug allergy is unknown in this context. Moreover, some authors in the past hypothesized in favor of a possible role of allergen-specific immunotherapy (ASIT) as a trigger of disease.Objectives:Aim of the present study is to establish the prevalence of each category allergen sensitization and to determine whether atopy or specific immunotherapy could influence clinical expression of the disease.Methods:Our study consisted in a retrospective demographic and clinical data collection regarding EGPA history (including age at diagnosis, organ and tissue involvement, autoantibody profile) and the presence of allergic comorbidities or previous drug hypersensitivity reactions. Patients without either proven allergic reactions or positive tests have been excluded.Results:Fifty-three (53) patients with definitive diagnosis of EGPA have been included in the analysis among which 25 (47.2%) with chronic respiratory allergy or previous acute allergic reaction. Among allergic patients 15 (60%) resulted sensitized towards inhalants and among them 13 (86.7%) displayed multiple sensitization. Drug allergy affected 13 patients (52%), food 4 (16%). Among 15 patients with respiratory allergy, 13 were eligible to allergen-specific immunotherapy (ASIT). Seven (7) subjects underwent ASIT prior EGPA diagnosis with an average time-to-EGPA of 16.2 years. No statistically significant difference was found in terms of sex, age at diagnosis, positivity for or specificity of anti-neutrophil cytoplasm antibodies (ANCA), eosinophil count at onset, pattern of clinical manifestations comparing allergic vs. non-allergic, ASIT vs. non-ASIT, ASIT vs. allergic, ASIT vs. eligible.Conclusion:Among patients with EGPA allergies are highly prevalent, particularly towards inhalants and drugs. In the great majority of patients multiple sensitization profile is found. Atopy doesn’t seem to be associated with specific patterns of disease presentation. The absence of correlation between inhalant ASIT exposure and variation in mode and time of EGPA onset doesn’t support the hypothesis of a its potential role in triggering the disease.References:[1]Berti A et al. Severe/uncontrolled asthma and overall survival in atopic patients with eosinophilic granulomatosis with polyangiitis. Respiratory Medicine 2018; DOI: 10.1016/j.rmed.2018.07.017[2]Cottin V et al. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg–Strauss). European Respiratory Journal 2016; DOI: 10.1183/13993003.00097-2016Disclosure of Interests:Luca Moroni: None declared, adriana cariddi: None declared, Silvia Sartorelli: None declared, Emanuel Della Torre: None declared, Tommaso Germanò: None declared, Giuseppe Alvise Ramirez: None declared, Enrica Bozzolo: None declared, Mona-Rita Yacoub: None declared, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

Background:Myocarditis is an infrequent but potentially life-threatening inflammatory disorder and might be part of the spectrum of systemic lupus erythematosus (SLE). Little is known about the clinical and histologic features of myocarditis in SLE, especially compared to other forms of myocarditis.Objectives:to test for potential distinctive traits among myocarditis in SLE (MyoSLE), SLE without myocarditis (OnlySLE) and myocarditis without SLE (OnlyMyo)Methods:Patients with MyoSLE were identified from three centres and compared with 231 cross-sectionally enrolled patients with OnlySLE and 87 patients with OnlyMyo. MyoSLE patients were split into two groups based on myocarditis onset within (early onset) vs after (late onset) the first year from SLE diagnosis. OnlySLE patients were dichotomised in the same way based on disease duration at time of enrolment. Demographics and general clinical features were collected retrospectively. SLE disease activity index 2000 (SLEDAI-2K), SLE International Collaborating Clinics/American College of Rheumatology damage index (SDI), clinical and laboratory features were collected at time of myocarditis onset in MyoSLE and at enrolment in OnlySLE. Quantitative data are expressed as median [interquartile range].Results:Fourteen MyoSLE patients were identified, 50% with early onset. Women were equally frequent among MyoSLE (71%) and OnlySLE patients (87%) and less frequent in the OnlyMyo group (43%; pConclusion:Demographics of patients with MyoSLE are more similar to patients with OnlySLE than to OnlyMyo patients. MyoSLE might have distinct histological and pathogenic features compared to OnlyMyo. Patients with MyoSLE show similar patterns of disease activity and accrued damage at time of myocarditis onset compared to patients with OnlySLE with the same disease duration but might diverge later on in SLE course. aPL are frequent in MyoSLE and might both contribute to the pathogenesis of myocardial inflammation and account for the high prevalence of NPSLE and APS, especially in late onset cases.References:[1]Gartshteyn Y et al., Lupus, 2020[2]Thomas G et al., J Rheumatol, 2017[3]Peretto G et al., Int J Cardiol, 2019[4]McDonnell T et al., Blood Rev, 2019Disclosure of Interests:Giuseppe Alvise Ramirez: None declared, Maria Gerosa: None declared, Giacomo De Luca Speakers bureau: SOBI, Novartis, Celgene, Pfizer, MSD, Lorenzo Beretta Grant/research support from: Pfizer, Simone Sala: None declared, Giovanni Peretto: None declared, Luca Moroni: None declared, Francesca Mastropaolo: None declared, adriana cariddi: None declared, Silvia Sartorelli: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Enrica Bozzolo: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

Background:Subglottic stenosis (SGS) is defined as airway narrowing below the vocal cords and is a common and potentially life-threatening manifestation of Granulomatosis with Polyangiitis (GPA), with an estimated prevalence of 16-23% (1). Balloon catheter dilation is effective in GPA-related SGS, but relapses are frequent. Little is known about the role of immunosuppression in this setting.Objectives:to analyse the clinical characteristics of a monocentric GPA cohort, describe phenotype differences among patients with and without SGS and investigate the role of surgical and medical treatments on relapse risk and general outcome.Methods:Biopsy-proven patients with SGS were identified by review of medical charts among a cohort of patients with GPA, classified according to the algorithm of the European Medicine Agency (2). The clinical characteristics of patients with SGS were retrospectively collected over a median follow-up time of 15.9 years and compared to those of patients without SGS.Results:Fourteen patients with SGS-GPA were identified, with a female to male ratio of 1:1 and a prevalence of 29.2% among the cohort. The mean ± SD age at GPA onset was 30.8 ± 14.4 years, with a mean time from GPA diagnosis to SGS onset of 4.7 ± 4.2 years. ANCA were positive in 78.6% (54.0% anti-PR3, 18.1% anti-MPO and 27.9% IFI only). The mean Birmingham Vasculitis Activity Score (BVAS) at onset was 10.0 ± 5.6. The main clinical manifestations associated with SGS were crusty rhinitis (100%), sinusitis (78%), pulmonary disease (72.7%), otitis/mastoiditis (50%), glomerulonephritis (42.9%), orbital pseudotumor (28.6%). Six patients (42.9%) received medical treatment only, other six (42.9%) had one to three balloon dilations and two (14.2%) underwent four or more procedures. Eight patients had no SGS relapse (maximum one dilation) and they all received immunosuppression with rituximab (RTX), cyclophosphamide (CYC) or azathioprine (AZA). All patients who received no immunosuppression, methotrexate (MTX) or mycophenolate (MMF) had at least one relapse. Patients treated with MTX or MMF had a mean relapse-free survival of 13.1 months, which was comparable to the one of patients not receiving medical treatment (40.2 months; p=NS) and shorter than the one of patients receiving CYC or RTX (153.2 months; p=0.032). CYC use also inversely correlated with the number of surgical procedures (r=-0.691, p=0.006). Compared to patients without SGS (31 consecutive patients with at least 4 years of follow-up), patients with SGS-GPA had an earlier disease onset (mean age 30.8 vs 50.4 years; pConclusion:Subglottic stenosis is highly prevalent in patients with GPA and may define a milder disease subset occurring more frequently in younger patients. MTX and MMF might be insufficient to prevent SGS relapses requiring balloon dilation. Aggressive immunosuppression (CYC or RTX) might have a non-redundant role in this setting and reduce the risk of relapses.References:[1]Quinn KA, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology 2019; 58 (12), 2203-2211.[2]Watts R, et al. Development and validation of a consensus methodology for the classification of the ANCA associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-7.Disclosure of Interests:Luca Moroni: None declared, Laura Giudice: None declared, Giuseppe Alvise Ramirez: None declared, Silvia Sartorelli: None declared, adriana cariddi: None declared, Angelo Carretta: None declared, Enrica Bozzolo: None declared, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

Background:Methotrexate (MTX) represents theanchor drugfor the treatment of rheumatoid arthritis (RA), as well as other rheumatological diseases such as psoriatic arthritis (PsA) and spondyloarthritides (SpA). Despite consolidated clinical efficacy, the use of MTX suffers from relevant limitations. Common issues include subjective intolerance, nausea, malaise, and fatigue, which negatively impact on quality of life and work/social participation.Objectives:In this study, we evaluated the frequency with which patients on MTX therapy opt for self-administration over the weekend or in the evening hours, in order to minimize interference with daily activities, work productivity, and social participation.Methods:A cross-sectional, prospective study was performed in two tertiary referral Rheumatology clinics, which included consecutive patients with RA, PsA, or SpA on MTX therapy. Enrolled patients had not previously received instructions by their healthcare provider as to when during the week or day MTX ought to be administered, and were free to choose or change the weekday and time for self-administration. Data on the route and timing of MTX self-administration was collected using dedicated questionnaires, which included queries on the dose and route of administration, day of the week and time of self-administration, reasons for the patient’s choice, use of folic acid supplementation, and concomitant therapies. Statistical analyses were conducted using a chi-square test; a p-value Results:A total of 275 consecutive patients treated with MTX were included, mostly with RA (86%). Patients had an average age of 59.8 years (SD 14.0) and an average disease duration of 134.5 months (SD 127.3). The average MTX dose was 15 mg/wk (SD 4.4); MTX was administered subcutaneously in 68.2% of cases, orally in 17.8%, and intramuscularly in 5%. Regarding the timing of MTX self-administration, 157 patients (57.1%) took MTX in the evening and 119 patients (44.3%) took it during the weekend; even among patients taking MTX in the evening, weekend administration was preferred (93/157, 59.2%, p Conclusion:The majority of patients with inflammatory arthritis opt for self-administration of MTX in the evening (absolute majority) or during the weekend (relative majority). This choice is dictated by the need to avoid side effects and detrimental repercussions on the individual’s social or working life. The adoption of these strategies for minimizing the adverse effects of MTX is more frequent among younger patients, and provides an indirect, yet powerful indicator of the impact of MTX therapy on patients’ quality of life.Disclosure of Interests:Giulio Cavalli Consultant of: SOBI, Pfizer, Sanofi, Novartis, Paid instructor for: SOBI, Novartis, Speakers bureau: SOBI, Novartis, Martina Biggioggero: None declared, adriana cariddi: None declared, Giacomo De Luca Speakers bureau: SOBI, Novartis, Celgene, Pfizer, MSD, Elena Agape: None declared, nicola boffini: None declared, Elena Baldissera Speakers bureau: Novartis, Pfizer, Roche, Alpha Sigma, Sanofi, Lorenzo Dagna: None declared, Ennio Giulio Favalli Consultant of: Consultant and/or speaker for BMS, Eli-Lilly, MSD, UCB, Pfizer, Sanofi-Genzyme, Novartis, and Abbvie, Speakers bureau: Consultant and/or speaker for BMS, Eli-Lilly, MSD, UCB, Pfizer, Sanofi-Genzyme, Novartis, and Abbvie

Background Diffuse alveolar haemorrhage (DAH) is a rare and severe manifestation of ANCA-associated vasculitides (AAV). Objectives To identify predictors of survival in patients with AAV-DAH. Methods A retrospective study of 102 consecutive patients (50% females; mean age 59±17 years) from 27 Italian Centres diagnosed with AAV-DAH was planned. Cox regression unadjusted analyses were performed. Results Among AAV patients, 47% had Granulomatosis with Polyangiitis (GPA), 47% Microscopic Polyangiitis (MPA) and 6% Eosinophilic Granulomatosis with Polyangiitis (EGPA). At DAH onset, mean BVAS was 20±8 and most patients had renal involvement (RI). Admission to Intensive Care Unit was needed in 27% of patients, while ventilatory support (VS) was required in 46%. At least one cardiovascular risk factor (CVRF) was recorded in 48%. Over a median follow-up of 39 months (25%–75% IQR 66 months), 19/102 patients (18.6%) died (figure 1). All patients received high-dose glucocorticoids in association with Cyclophosphamide (CYC 78%, mean cumulative dose 8±7 g) or Rituximab (37%). Plasma exchange was performed in 46%. Infections occurred in 38%. Age>65 years (HR 3.05 [95% CI 1.18–7.9], p=0.04), CVRF ≥2 (HR 8.85 [95% CI [AG7] 2.34–33.50], p=0.01), BVAS (v.3) (HR 1.07 [95% CI 1.01–1.13], p=0.01) were associated with mortality, whereas FFS was not. The need for VS (HR 4.54 [95% CI 1.48–13.85], p=0.008) and infections (HR 3.98 [95% CI 1.48–10.69] were also associated with mortality. Conclusions Older age, VS, CVRF and infections affect the survival in AAV. There is a need for specific outcome measeures. Disclosure of Interest None declared

Background Takayasu arteritis(TA) is a large-vessel vasculitis affecting aorta and its major branches1. Vascular inflammation may cause luminal narrowing, occlusion or aneurysms2. TA affects more frequently young women, but occurrence in patients>40 years is not rare. Female:male ratio is 9:13 Objectives To compare age at disease onset, age at diagnosis and diagnostic delay between male and female Italian TA patients from our cohort. To compare symptoms presentation and vascular involvement Methods Data from 10 male and 104 female TA patients (fulfilling ACR criteria) from our tertiary centre were retrospectively collected. Age at diagnosis, age at first symptom onset, diagnostic delay, signs and symptoms subsequently attributed to TA, and arteries involved before the diagnosis were compared. Non-parametric statistic tests were used Results In male patients mean age at diagnosis was 33.5±14.64 years and mean age at onset was 25.8±13.01 years. In female patients mean age at diagnosis was 36.26±12.6 years and mean age at onset was 31±13.1 years. The differences not being statistically significant. The highest percentage of male patients disease onset was Conclusions Male patients with TA have more frequent involvement of the coronary arteries and of the iliac and femoral arteries. As a consequence, they suffer more frequently acute coronary syndrome and lower limbs claudication. Besides, they have more frequently arterial hypertension References [1] Numano F, et al. Takayasu arteritis. Lancet2000. [2] Clomarmond C, et al. Long-Term Outcomes and Prognostic Factors of Complications in Takayasu Arteritis. Circulation2017. [3] Fatos O, et al. Epidemiology of Takayasu arteritis. Presse Med2017. Disclosure of Interest A. Tomelleri: None declared, C. Campochiaro: None declared, S. Sartorelli: None declared, A. Cariddi: None declared, G. Cavalli: None declared, E. Baldissera: None declared, L. Dagna Grant/research support from:>>The Unit has received unrestricted educational grants from Abbvie, BMS, Celgene, Mundipharma, Novartis, MSD, Pfizer, Roche, and SOBI.

Despite being the second least represented granulocyte subpopulation in the circulating blood, eosinophils are receiving a growing interest from the scientific community, due to their complex pathophysiological role in a broad range of local and systemic inflammatory diseases as well as in cancer and thrombosis. Eosinophils are crucial for the control of parasitic infections, but increasing evidence suggests that they are also involved in vital defensive tasks against bacterial and viral pathogens including HIV. On the other side of the coin, eosinophil potential to provide a strong defensive response against invading microbes through the release of a large array of compounds can prove toxic to the host tissues and dysregulate haemostasis. Increasing knowledge of eosinophil biological behaviour is leading to major changes in established paradigms for the classification and diagnosis of several allergic and autoimmune diseases and has paved the way to a “golden age” of eosinophil-targeted agents. In this review, we provide a comprehensive update on the pathophysiological role of eosinophils in host defence, inflammation, and cancer and discuss potential clinical implications in light of recent therapeutic advances.