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BackgroundIn women with Hereditary Angioedema (HAE) due to C1-inhibitor (C1INH) deficiency (C1INH-HAE), pregnancy counseling and treatment can be challenging. Despite the evidence of the immediate favorable outcome and safety of plasma-derived (pd)C1INH concentrate, there are no data regarding any difference among women who underwent or not pdC1INH during pregnancy or on children with in utero exposure to pdC1INH. The present interview study aimed at analyzing outcome of C1INH-HAE mothers and children according to pdC1INH-exposure during pregnancies.MethodsC1INH-HAE women who experienced at least 1 pregnancy were included from seven centers of the Italian Network for Hereditary and Acquired Angioedema (ITACA). The interview study retrospectively analyzed pregnancies who underwent (group 1) or not (group 2) pdC1INH. The overall goals of the study included immediate and long-term outcomes, in terms of outcomes in the time interval between pregnancy and survey.ResultsA total of 168 pregnancies from 87 included women were analyzed. At term delivery (>37 gestation-week, GW) has been registered in 73.8% of cases, while spontaneous abortion (SA) occurred in 14.2% of cases with a mean GW 7 ± 2. The group 1 including pdC1INH-treated pregnancies comprised a third of the cohort (51/168, time interval 1.5 ± 10.4 yrs), while the group 2 represented 69.6% (117/168, time interval 32.8 ± 14 yrs). The same prevalence of SA occurred when comparing group 1 (11.7%) with group 2 (15.4%) with a similar GW at SA. The group 1 was older at the pregnancy time and younger at the interview than the group 2 (P < 0.01 for both); moreover, the group 1 showed a higher prevalence of cesarean delivery (P < 0.0001). The overall prevalence of obstetrical syndromes was similar between two groups: however, gestational diabetes was described only in pdC1INH-untreated pregnancies. In utero pdC1INH-exposed children (n = 45) did not show differences compared with unexposed ones (n = 99) in neonatal short-term outcomes.ConclusionThrough appropriate management and counseling, most of C1INH-HAE women undergo successful pregnancy and delivery. For pregnant C1INH-HAE women being treated with pdC1INH, our findings are reassuring and might lead to an improvement of both the knowledge about treatments and the experience of HAE itself.

BackgroundDermatomyositis (DM) is a rare idiopathic inflammatory myopathy that can present with a variable spectrum of cutaneous and systemic manifestations1. Correlations between myositis-specific autoantibodies (MSAs) and disease phenotype have been documented in DM in particular in association with the development of interstitial lung disease (ILD). Ongoing studies focus on the potential role of MSAs in similarities of the pathophysiological mechanisms of COVID-19 associated ILD and that related to DM suggesting an intriguing cross talk between autoimmunity and COVID-192.ObjectivesTo explore clinical patterns including ILD and MSAs profile in DM patients from a population-based single-center study carried out in a Tertiary Referral Rheumatologic Clinic. SARS-CoV-2 infections and vaccination were also analysed.MethodsWe enrolled patients affected by DM classified according to 2017 EULAR/ACR criteria1 with a disease onset at ≥18 years referring to the Rheumatologic Clinic of Tor Vergata University Hospital in Rome (Italy). Clinical data were collected from medical records: gender, age of onset of symptoms and diagnosis, clinical features, auto-antibodies patterns (ANA, MSAs including anti-tRNA synthetase, -Jo1, -PL7, -EJ, - MDA5, -NXP2, SAE, Mi2, and myositis-associated antibodies comprising anti-PM/Scl, -Ro-52, -Ku, U1RNP), pattern of lung abnormalities at thoracic CT scans, and treatments. The prevalence of SARS-CoV-2 infection and vaccination profile were also investigated.ResultsAmong eligible cases (n=30), patients who completed the study (n=19) included almost entirely women (F=73.7%). The median age at disease onset was 57.4±12.4 yrs while the mean diagnostic delay resulted as 12±10 months. Skin manifestations and myalgia were the prevalent symptoms (79% and 63%, respectively) whereas dyspnea and cough occurred in a third of the cohort at DM onset. Besides the skin involvement, the decrease in pulmonary function was the main clinical manifestation at the first rheumatological referral (52.6% of cases) followed by joint pain (36.8%) and cardiovascular events (10.6%). ANA titer≥1:160 occurred in 79% of patients. All patients showed MSAs positivity with a similar distribution: a single case of double positivity was registered in a man with anti-MDA5 and -NXP2. The most common CT findings were ground-glass opacity and parenchymal band in a third of patients (32%). The entire cohort had undergone cycles of steroids from the onset of DM and during the follow-up, according to disease severity. The main used therapies were methotrexate (47%), intravenous immunoglobulin (42%) and mofetil micofenolate (31.6%). None of patients had SARS-CoV-2 infection (until December 2021). The 63% of the cohort received at least one dose of the anti- SARS-CoV-2 mRNA vaccine (BNT162b2), of which 10% had completed with the booster dose. No adverse reactions or post-vaccination DM flare were registered. A third of the cohort had not been vaccinated due to concomitant disease activity or therapies. No cases of post-COVID19 new-onset DM were diagnosed.ConclusionOur preliminary findings support the relevant impact of lung involvement in DM. The availability of MSAs can help to stratify patients with DM for outcome and risk of potential disease complications. The impact of MSAs on ILD associated or not to COVID-19 deserve further investigations in a larger DM cohort and for a longer post-COVID-19 pandemia follow-up time.References[1]Lundberg IE, et al. 2017 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. Arthritis Rheumatol. 2017;69:2271-82.[2]Wang Y, et al. Similarities and differences between severe COVID-19 pneumonia and anti-MDA-5-positive dermatomyositis-associated rapidly progressive interstitial lung diseases: a challenge for the future. Ann Rheum Dis. 2020:annrheumdis-2020-218594.Disclosure of InterestsNone declared

BackgroundInterstitial lung disease (ILD) in Rheumatoid Arthritis (RA) can be subclinical or underestimated. Data from the literature report male sex, seropositivity and old age as known risk factors for ILD in RA. However, the correct timing for referral to pneumologists and eligibility for CT chest scan is undefined.ObjectivesTo identify potential red flags of ILD diagnosis for referral to a multidisciplinary team analyzing clinical and radiological findings associated with the establishment of ILD.MethodsRetrospective study on CT chest scan from RA patients evaluated and categorized into usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP) and unclassifiable RA-ILD (Un-ILD), by two radiologists. RA-ILD was diagnosed by two pneumologists considering radiological and clinical factors.Results80 CT chest scans from RA patients (F 59, age 66,6 ± 11,4, disease duration 7,8 ± 4,4) were evaluated. 36,5% of patients had performed CT-chest scan for suspect of ILD, 11,2% for probable pulmonary infection, 8,7% for oncological screening or follow up, 13,7% for COPD disease, 8,7% for screening or active TBC, 3,7% for screening or previous pleuritis and 17,5% for other reasons. A RA-ILD pattern was identified in 35% of patients, with a defined UIP pattern in 17,8%, a defined NSIP pattern in 42,8% and a 39,3% of Un-ILD, while 65% of patients had a non-ILD lung involvement by CT-scan. The mean age at ILD diagnosis was 67,2 years and the mean time between RA diagnosis and onset of ILD symptoms was 7,4 years.In the multivariate analysis female sex (HR=0,33; p=0,006) and disease duration (HR=0,8; p=0,06) were protective factors for ILD diagnosis. Age (HR=1,1; p=0,03), use of leflunomide (HR=10,1; p=0,03) were negative prognostic factors for ILD. Pulmonary clinical symptoms as dry cough (HR=17,6; p=0,02), bibasilar inspiratory crackles (HR=11,5; p=0,01) and pleural rubs (HR=18,1; p=0,03) predicted ILD while negative predictors were the presence of linear opacities (HR=99,9; p=0,006), interstitial abnormalities (HR=12,1; p=0,07) and cystic areas (HR=33,3; p=0,06) at chest CT scan.ConclusionIn our cohort the first ILD symptom appeared 7.4 years after RA diagnosis. Clinical variables and radiological abnormalities were identified as highly predictive of ILD diagnosis and may represent red flags for an early diagnosis and referral to pneumologists and radiologists in a multidisciplinary approach.Disclosure of InterestsNone declared

BackgroundPsoriatic Arthritis (PsA) is a chronic inflammatory disease, characterized by both articular and periarticular manifestations, usually associated with psoriasis. The identification of the correct therapy for patients is still a critical issue, but the use of biological drugs, such as TNFi (Tumor Necrosis Factor Inhibitors), modified the outcome of PsA patients even if there is great variability in the clinical efficacy. Since the response to drugs is a complex trait, the identification of genetic factors could help to define new genomic biomarkers for more effective and personalized therapy.ObjectivesThe aim of this study was to evaluate the potential role of polymorphisms in genes already known to be involved in PsA susceptibility (ERAP1, HCP5, IL10, MIR146, PSORS1C1, STAT4, TNFAIP3 and TRAF3IP2) as predictors of efficacy of treatment, in a cohort of Italian PsA patients, treated with first-line TNF-i, in particular with Etanercept (ETN) and Adalimumab (ADA).MethodsPolymorphisms were analyzed in a cohort of 163 patients with peripheral PsA. For each patient was estimated the Disease Activity in Psoriatic Arthritis (DAPsA) score. Genotyping was performed by allelic discrimination by TaqMan assay. The possible association between the selected SNPs and mean values of DAPsA differences, at 22 (ΔT22) and 54 (ΔT54) weeks from the beginning of the TNF-i treatment, were evaluated by T-test. A multivariate logistic regression analysis was used to evaluate the contribution of each genetic variant investigated in the TNF-i treatment response.ResultsWe have observed that TRAF3IP2 SNP was associated with TNF-i treatment response in PsA patients. In particular, the patients carrying variant alleles seem to respond better to treatment, both at 22 (P = 0.032) and 54 weeks (P = 0.019). Moreover, the variant allele of TRAF3IP2 SNP resulted associated with a better response of joints involvement. Indeed, the number of tender and swollen joints decrease more in patients carrying variant allele (P = 0.006 and P = 0.024, respectively). We also observed that PsA patients carrying IL10 variant allele decrease their mean DAPsA value less than patients with wild-type genotype only at 54 weeks of treatment (P = 0.031). Also, HCP5 polymorphism showed a difference of mean difference of DAPsA values between genotypes for both follow up, even if these difference does not reach a statistical significance (P = 0.068 and P = 0.086). The multivariate regression analysis was performed with a stepwise method and it confirmed the involvement of TRAF3IP2 (P = 0.016) and HCP5 (P = 0.035) polymorphisms in the TNF-i response after 22 weeks and of TRAF3IP2 (P = 0.007), IL10 (P = 0.022) and HCP5 (P = 0.036) polymorphisms after 54 weeks. The two final models explain about 6% and 11% of the variability in TNF-i treatment response at 22 and 54 weeks respectively.ConclusionOur results suggest that some polymorphisms in genes associated to PsA susceptibility could also play a role in TNFi treatment response and could give a contribution in the definition of a genetic profile associated with the response to anti-TNF drugs.Figure 1.Mean differences of DAPsA values in the genotypic classes for TRAF3IP2, IL10 and HCP5 SNPs. DAPsA = Disease Activity Index for PsA; Wt = wild-type genotype; Hz = heterozygous genotype; Homo var = homozygous variant genotype.Disclosure of InterestsNone declared

BackgroundAxial Spondyloarthritis (axSpA) can be distinguished in radiographic axSpA (r-axSpA) and non-radiographic (nr-axSpA). Secukinumab (SEC) is a novel treatment for axSpA, but data from real-life are still missing.ObjectivesTo evaluate, in a multicentric-Italian-cohort of axSpA patients on SEC followed for 24-months: a)the long-term effectiveness and safety of SEC; b)the drug-retention-rate and low-disease-activity (LDA) measured as BASDAIMethodsConsecutive patients with active axSpA (diagnosis according Assessment of SpondyloArthritis International Society ASAS criteria), who started SEC treatment, were evaluated prospectively. Data on disease characteristics, previous/ongoing treatments and imaging were collected. Disease-activity/functional/clinical scores and biochemical values were recorded at baseline (T0), at 6 (T6), 12 (T12), and 24 (T24) months. Effectiveness was evaluated over-time with descriptive statistics; multivariate Cox and logistic regression models were used to evaluate predictors of drug-discontinuation and LDA at T6. Infections, adverse events were recorded.Results249 patients (47.8% male; median age 51 years) were enrolled; 40.9% had HLA-B27; 53.8% had r-axSpA and 46.2% nr-axSpA. SEC was prescribed in 28.9% naïve and in 71.1% non-naïve patients. SEC effectiveness was shown as an improvement in several outcomes, such as ASDAS [T0=3.5(2.9-4.4) vs. T24=1.9(1.2-2.4);p=0.02] and BASDAI [T0=6.5(5.0-7.5) vs. T24=2.8(1.8-4.0);p=0.03]. After 24-months of treatment, 90.7% of naïve and 75.3% of non-naïve patients achieved LDA (BASDAITable 1.Clinical, functional, disease activity and serological parameters of naïve (n=72) and non-naïve (n=177) axSpA patients during the 24-month follow-upT0T6T12T24BASMI [0-10], median (IQR)naïve2.0 (1.0-4.0)2.0 (0.0-3.3)1.0 (0.0-2.8)1.0 (0.0-2.0)non-naïve4.0 (1.0-7.0)4.0 (1.0-7.0)2.0 (1.0-4.0)2.0 (1.0-3.0)pp=0.03p=0.04nsnsHAQ-S [0-8], median (IQR)naïve0.8 (0.5-1.3)0.5 (0.0-1.0)0.3 (0.0-1.0)0.1 (0.0-0.5)non-naïve1.1 (0.8-1.5)1.0 (0.4-1.3)0.6 (0.4-0.9)0.7 (0.1-0.9)pp=0.04p=0.04p=0.04p=0.04ASDAS [0-6], median (IQR)naïve3.3 (2.7-3.9)2.1 (1.6-2.7)2.0 (1.2-2.8)1.3 (1.0-2.2)non-naïve3.7 (2.9-4.7)2.9 (2.0-3.7)2.1 (1.9-3.1)2.3 (1.6-2.3)pnsnsnsp=0.04ESR [0-25](mm/h), median (IQR)naïve14.0 (6.0-27.0)10.0 (4.0-19.5)8.0 (3.1-12.8)5.0 (2.0-15.0)non-naïve18.5 (10.0-31.3)15.0 (8.0-25.0)12.0 (6.0-20.0)12.0 (6.0-19.0)pp=0.04p=0.04p=0.04p=0.04Legend: BASMI: Bath Ankylosing Spondylitis Metrology Index; HAQ: Health Assessment Questionnaire modified for spondyloarthritis; ASDAS: Ankylosing Spondylitis Disease Activity Score; ESR: erythrocyte sedimentation rateConclusionIn a real-life clinical-setting, SEC proved to be safe and effective in axSpA, mainly in naïve-patients, with a notable drug-retention-rate. No differences were observed between r-axSpA and nr-axSpA.AcknowledgementsContributing authors of the Spondyloartritis and Psoriatic Arthritis SIR Study Group “Antonio Spadaro”: Alberto Cauli, MD, PhD, Rheumatology Unit, Department of Medical Sciences, AOU and University of Cagliari, Cagliari, Sardegna, Italy; Angelo Semeraro, MD, Rheumatology Unit, Martina Franca-ASL Taranto, Puglia, Italy; Leonardo Santo, MD, Rheumatology Unit, ASL BT Andria – DSS4 Barletta, Italy, Barletta-Andria-Trani, Puglia, Italy; Emanuela Praino, MD, Rheumatology Unit, ASL BT Andria – DSS4 Barletta, Italy, Barletta-Andria-Trani, Puglia, Italy; Giorgio Amato, MD, Rheumatology Unit, A.O.U. Policlinico S. Marco, Catania, Sicilia, Italy; Nicolò Girolimetto, MD, Rheumatology Unit, Department of Internal Medicine, Azienda USL-IRCCS, Istituto di Ricovero e Cura a Carattere Scientifico, Reggio Emilia, Emilia Romagna, Italy.Disclosure of InterestsGiacomo Cozzi: None declared, Mariagrazia Lorenzin: None declared, Maria Sole Chimenti: None declared, Salvatore D’Angelo: None declared, Antonio Marchesoni: None declared, Carlo Salvarani: None declared, Ennio Lubrano: None declared, Luisa Costa: None declared, Ylenia Dal Bosco: None declared, Elena Fracassi: None declared, Augusta Ortolan: None declared, Mario Ferraioli: None declared, Antonio Carriero: None declared, elisa visalli: None declared, RICCARDO BIXIO: None declared, Francesca Desiati: None declared, ALBERTO BERGAMINI: None declared, elisa pedrollo: None declared, Andrea Doria Grant/research support from: Novartis, Abbvie, Pfizer, MSD, Janssen, Rosario Foti: None declared, Antonio Carletto: None declared, Roberta Ramonda Grant/research support from: Novartis, Abbvie, Pfizer, MSD, Janssen

BackgroundMicronutrients play immunomodulatory roles through interactions with innate and adaptive immunity influencing the pro/anti-inflammatory balance. Serum levels depend on multiple factors such as gender, nutrition, and gut microbiome. Micronutrient deficiencies (MNDs) are associated with a pro-inflammatory status and co-morbidities in patients with chronic inflammatory diseases. No studies focused on potential interplay between MNDs and disease phenotype in Enteropathic Spondyloarthritis (ESpA) in which the combination of SpA with inflammatory bowel diseases (IBD) might dramatically affect micronutrients status.ObjectivesWe analysed the occurrence of anemia (Hb ≤12 g/dl) and deficiencies of ferritin (Fe, ≤15 ng/dL), vitamin D [25(OH)D, ≤20 ng/ml], vitamin B12 (VB12, ≤200 pg/ml), and folic acid (FA, ≤ 4ng/mL) in ESpA patients. The interplay of MNDs with gender, metabolic parameters, HLA-B27 susceptibility, type of SpA and IBD, disease activity, and treatments was also explored.MethodsSelection criteria of this cross-sectional descriptive study consisted of having a diagnosis of ESpA, regardless of its type, in an age of ESpA onset ≥18 and ≤80 years, among patients who were admitted to a combined Gastro-Intestinal and RHEumatologic “GI–Rhe” clinic (Tor Vergata University Hospital, Rome, Italy). Exclusion criteria were represented by active IBD, pregnancy or lactation, kidney and/or liver failure, alcohol abuse, neoplasia, ongoing supplementations. SpA disease activity was assessed by ASDAS-CRP and functional status by HAQ-S. All the enrolled patients underwent blood chemistry analysis to determine parameters including CRP, uric acid (SUA), Fe, 25(OH)D, VB12, FA, and HLA-B27 typing.ResultsWe included 164 patients comprising 109 females and 55 age-matched males. A diagnosis of Crohn’s Disease (CD) occurred in 70% of patients while Ulcerative Colitis (UC) represented a third of the cohort. Peripheral (per) and axial (ax) SpA were equally distributed (50%vs50%). Patients with ax-SpA displayed a greater prevalence of CD than UC (p=0.02) while UC was prevalent in per-SpA (p=0.02). B27+ was revealed in 19% of our cohort: B27+ patients had a higher prevalence of ax-SpA (p=0.016) and a more severe disease activity (p=0.02) than B27-. Moreover, B27 positivity and uveitis were prevalent in ax-SpA compared to per-SpA (p=0.009 and p=0.01, respectively). According to univariate analysis, males showed higher SUA (P=0.004) and BMI (p=0.03) than females. Conversely, females showed a higher prevalence of anemia than males (p=0.002). A third of ESpA cohort showed FA (31.6%) and 25(OH)D (27.8%) deficiency while VB12 defect was less frequent (18.2%) and was registered almost entirely in B27- ESpA (p=0.02). CD-ESpA showed a lower mean VB12 (p=0.04) and a higher prevalence of ocular/skin co-morbidities (p=0.02) and ax-SpA (p=0.04) than UC-ESpA. Accordingly, CD-ESpA were on bDMARDs more than UC-ESpA (p=0.04).ConclusionOur findings document that FA and 25(OH)D deficiencies represent the main MNDs among ESpA patients while VB12 seems to be deficient mostly in patients with CD and almost exclusively in B27- patients. Otherwise, B27+ in ESpA results to be associated mainly with disease phenotype and treatments. In ESpA, the gender of patients appears to impact principally on dysmetabolism highlighting the role for nutritional interventions particularly in males. The interplay of MNDs with B27 and dysmetabolism in ESpA deserves further investigations also taking into account CD/UC localization and behavior.References[1]Park YE, et al. Incidence and risk factors of micronutrient deficiency in patients with IBD and intestinal Behçet’s disease: folate, vitamin B12, 25-OH-vitamin D, and ferritin. BMC Gastroenterol. 2021;21(1):32.doi: 10.1186/s12876-021-01609-8.[2]Conigliaro P, et al. Impact of a multidisciplinary approach in enteropathic spondyloarthritis patients. Autoimmun Rev. 2016;15(2):184-90.doi: 10.1016/j.autrev.2015.11.002.Disclosure of InterestsNone declared

BackgroundJAK-inhibitors (JAK-i) represent an effective choice in achievement of therapeutic target in bio-naïve (bnaïve) and bDMARD-insufficient responders (bDMARD-IR) patients. However, oral glucocorticoids (OGC) are commonly used in clinical practice in combination with b/tsDMARDs in Rheumatoid and Psoriatic Arthritis (RA and PsA) patients in order to reach clinical remission.ObjectivesTo assess, in a real-life clinical setting, the reduction of OGC dose during JAK-i treatment in RA and PsA patients that were b-naïve or ≥1-bDMARD-IR or ≥2-bDMARDs-IR with different mechanisms of action.MethodsPatients with active RA or PsA treated with first JAKi were prospectively enrolled in a single center. Disease activity (DAS28-CRP or DAPSA), pain visual analogic scale (VAS-pain), general health (GH) and mean prednisone dosage (PDN dose) were collected at baseline and after 4, 8 and 12 months (T4, T8, T12) of treatment.Results89 patients with active arthritis (77 RA, 12 PsA) were treated with JAK-i (43 patients received Baricitinib, 41 Tofacitinib and 5 Upadacitinib), with all patients reaching T4, 65 patients T8 and 50 patients T12 (Table 1). 27% patients were b-naïve, 73% ≥1-bDMARD-IR and 34% ≥2-bDMARDs-IR. A mean OGC dose of 4.0 ± 4.6 (mg per day) was used and 39% of patients received JAK-i without csDMARDs. We observed a significant improvement of disease activity by DAS28-CRP or DAPSA, VAS-pain and GH at all time points in whole population (respectively pTable 1.Women75% (67)Age (years)60 ± 12Seropositive66% (51)Disease duration (months)132 ± 88DAS28 CRP4.0 ± 1.0DAPSA40.6 ± 19.7VAS-pain67.7 ± 15.8GH68.1 ± 15.2ConclusionJAK-i administration allows to reduce concomitant OGC regardless of line of b-DMARDs treatment. Chronic OGC may have detrimental bone, metabolic, cardiovascular and infective side effects; therefore JAK-i steroid-sparing effect may be beneficial for patients in long-term treatment.Disclosure of InterestsNone declared

BackgroundTumor necrosis factor (TNF)-α is one of principal proinflammatory cytokines involved in pathogenesis of different inflammatory arthropathies as Rheumatoid Arthritis (RA), Psoriatic Arthritis (PsA) and Ankylosing Spondylitis (AS). Biotechnological drugs, represented among others by monoclonal antibodies directed against TNF-α, lead to a revolution in RA and spondylarthritis treatment. They were the first biological drugs used to treat these diseases, with good results in terms of safety and efficacy. Also because of high cost of these drugs, for some years biosimilars have been introduced in clinical practice. Biosimilars are less expensive (in Italy, less of 30% than bioriginators); they underwent to a severe process of “comparability” to assess safety and efficacy like their originators. In Italy, AIFA authorized SB4 (etanercept biosimilar), SB5 and ABP501 (adalimumab biosimilars) after passing III-phase randomized clinical trials; but real-life data and registers are lacking.ObjectivesAim of this study is to compare biosimilars and bioriginators in terms of safety and efficacy in a real-life contest.MethodsWe consequently enrolled patients, affected by inflammatory arthropathies (RA, PsA, AS) and treated with biosimilars (SB4, ABP501), belonging to any of the main biological prescribing centers in the Lazio region, from 2017 to 2020. Moreover, we enrolled patients, affected by same inflammatory diseases, but treated with corresponding originator. Clinical and laboratory data as well as disease activity indices, were collected at recruitment (T0) and after 4 (T1), 8 (T2), 12 (T3) and 24 (T4) months of therapy. Adverse events were registered.ResultsThe multicenter cohort was composed by 455 patients treated with biosimilars (SB4/ABP501 276/179; F/M 307/146; naïve 56%, median age/IQR 55/46-65) and 436 treated with originators (etanercept/adalimumab 186/259, F/M 279/157, naïve 67,2%, median age/IQR 53/43-62). No differences were found about safety, but biosimilar group presented a higher number of discontinuations due to inefficacy (pConclusionReal-life data demonstrated the same safety between biosimilars and originators but a reduced biosimilar retention rate at 24 months, about 76%. Despite their loss efficacy, biosimilars could be considered valid and safe, and a good and less expensive alternative to the originators, allowing access to these innovative treatments to a wider patient population.References[1]Feldmann, M. & Maini, R. N. Anti-TNFα therapy of rheumatoid arthritis: what have we learned? Annu. Rev. Immunol. 2001;19, 163–196.[2]McInnes IB, Schett G (2007) Cytokines in the pathogenesis of rheumatoid arthritis. Nat Rev Immunol 7:429–442[3]Velayudhan J, Chen YF, Rohrbach A, Pastula C, Maher G, Thomas H, et al. Demonstration of functional similarity of proposed biosimilar ABP 501 to adalimumab. BioDrugs. 2016;30(4):339– 51.[4]Emery P, Vencovsky ́ J, Sylwestrzak A et al. Long-term safety and efficacy of SB4 (etanercept biosimilar) in patients with rheumatoid arthritis: comparison between continuing SB4 and switching from etanercept reference product to SB4. Ann Rheum Dis 2016; 75:236.Figure 1.Disclosure of InterestsNone declared

BackgroundPregnancy is a topic of fundamental importance for women living with autoimmune rheumatic diseases (ARD). Efforts at national and international levels have been put in the collection and harmonization of data in order to implement an evidence-based management of pregnant patients.ObjectivesThe P-RHEUM.it study was designed as a nationwide, web-based longitudinal observational cohort study to collect data about pregnancy in ARD in 26 centers in Italy. The study started in May 2018 and has been supported by the Italian Society for Rheumatology.MethodsPregnant patients with a definite rheumatic disease according international criteria were enrolled up to gestational week (GW) 20. The course of maternal disease activity, the use of medications, fetal and maternal complications, and the quality of life (EuroQoL questionnaire) were collected for each trimester, as well as pregnancy outcome, mode of delivery, neonatal complications, and maternal and children’s follow-up to 6 months after delivery, including the screening for post-partum depression by means of EPDS (Edinburgh Postnatal Depression Scale).ResultsAs of December 2021, 758 pregnancies had been enrolled, 205 (27%) ongoing and 553 (73%) with outcome. Pregnancy loss occurred in 54 (9.8%) cases (40 spontaneous miscarriages; 6 voluntary terminations). Live births were 495 (89.5%), perinatal death occurred in 4 (0.7%) cases. Table 1 reports on the group of 495 live births, along with subgroups of Rheumatoid Arthritis (RA) and Systemic Lupus Erythematosus (SLE), the two most represented diseases. Regarding treatments, 166 (30%) pregnancies were exposed to corticosteroids, 239 (43%) to hydroxychloroquine, 59 (10.7%) to csDMARDs, 84 (15.2%) to TNF inhibitors, 1 (0.2%) to non-TNFi bDMARDs, 299 (54%) to low dose acetylsalicylic acid, and 126 (22.8%) to heparin.Table 1.PREGNANCIES WITH LIVE BIRTHS, EXCLUDING PERINATAL DEATHSTotal pregnancies (n=495)RA pregnancies (n=69)SLE pregnancies (n=93)Age at conception (years)34 (31 - 37)34.5 (32 - 38)34 (31 - 36)Disease duration (years)6.1 (2.2 - 11.1)7.1 (4.3 - 11.6)9.3 (5.9 - 15.9)Caucasian431 (87.8%)53 (79.1%)75 (80.6%)Never smokers358 (73.8%)53 (80.3%)66 (71.7%)Body Mass Index >3045 (9.5%)7 (10.3%)5 (5.6%)Arterial Hypertension6 (1.2%)0 (0%)2 (2.2%)Time to pregnancy (months)3 (1 - 6)3 (1 - 6)3 (0 - 10)Physician-reported flares in the 12 months prior to conception107 (23%)22 (34.4%)13 (14.8%)Physician global assessment at enrolment (VAS 0-100)5 (0 - 17)5 (0 - 20)4 (0 - 10)Patient global health at enrolment (VAS 0-100)18 (7 - 30)10 (5 - 29)10 (5 - 25)EuroQoL at enrolment (-1.6 – 1)1 (0.8 - 1)1 (0.8 - 1)1 (0.8 - 1)Flares during pregnancy35 (7.1%)6 (8.7%)7 (7.5%)Hypertensive disturbances*8 (1.7%)1 (1.6%)6 (6.6%)Delivery at term (≥37 GW)410 (85.1%)53 (77.9%)74 (80.4%)Spontaneous vaginal delivery173 (35.9%)23 (33.8%)23 (25.3%)Congenital malformations11 (2.4%)2 (3.1%)1 (1.1%)Small for gestational age (SGA) neonate24 (4.9%)1 (1.4%)9 (9.9%)Breastfeeding in the first 4 weeks after delivery341 (79.7%)45 (77.6%)59 (76.6%)EPDS score at risk for post-partum depression22 (14.1%)0 (0%)3 (10.3%)Continuous variables are expressed as median (interquartile range); *gestational hypertension/preeclampsia/HELLP syndrome/eclampsia.ConclusionMultiple factors may have contributed to the high rate of live births, including good disease control before and during pregnancy thanks to the use of anti-rheumatic drugs and low frequency of general risk factors. SLE pregnancy was affected by a higher frequency of complications (hypertensive disturbances, SGA babies) as compared to RA pregnancy. Nearly 80% of patients breastfed in the first month after delivery. For the first time, data about the screening questionnaire for post-partum depression were collected, showing at least 1 out 10 patients can be at risk.References[1]Meissner Y et al. Arthritis Res Ther;21(1):241; Ann Rheum Dis. 2021;80(1):49-56.AcknowledgementsP-RHEUM.it study is supported by the Italian Society for Rheumatology (SIR). All the Investigators are acknowledged for their contribution.Disclosure of InterestsNone declared

BackgroundThe noninfectious uveitis (NIU) is the intraocular inflammation that can be the first or one of extra-articular manifestations of systemic inflammatory rheumatic diseases mainly including spondyloarthritis (SpA), Behcet’s disease (BD) and juvenile idiopathic arthritis (JIA)1. Rheumatologists are often asked to help in diagnostic assessment and relatively targeted treatment of particular subsets of NIU2.ObjectivesTo explore patterns and treatments of NIU from a population-based single-center study carried out in a Tertiary Referral Rheumatologic Clinic.MethodsSelection criteria of this cross-sectional descriptive study consisted of having a diagnosis of NIU in an age of onset ≥16 years old regardless of its etiology among patients who were admitted (February 2018-December 2021) to Rheumatologic Clinic of Tor Vergata University Hospital (Rome, Italy). Clinical data were collected from medical records: gender, age of onset and admission, recurrence and anatomic patterns, HLA-B27 susceptibility, associated rheumatologic diagnosis and treatments were investigated.ResultsWe included 100 patients with 58% women. Median age at NIU onset was 39.6±15 years whereas patients were 44±14 years old at the first referral, without gender-difference. NIU represented the main reason of referral in 22% of cases. Anterior uveitis occurred in 73% of patients and posterior in 25%; intermediate (IU) and panuveitis (PanU) were isolated cases. The prevalent course was acute (57%) followed by recurrent (31%) and chronic (12%); both eyes were involved in 39 patients. Almost half of NIU was associated with SpA including IBD-SpA (20%), ankylosing spondylitis (AS, 18%), and Psoriatic Arthritis (12%). A diagnosis of BD was reported in 14% of NIU while Rheumatoid Arthritis and other systemic autoimmune diseases occurred in 11% and 12%, respectively. Idiopathic NIU and JIA-NIU constituted the lowest prevalence (8% and 5%, respectively). A higher prevalence of BD-NIU and AS-NIU resulted in males than females (p+: a higher percentage of unilateral (p=0.09) and acute course (p=0.02) occurred in B27+ than B27-. AS was prevalent in B27+ NIU than B27- (p- NIU than B27+ (p=0.09 and p=0.04 respectively). The only two cases of IU and PanU were both idiopathic B27- NIU. At the onset of NIU, most patients were not on systemic treatment (68%): remaining cases were on cDMARDs (22%) and bDMARDs (10%). During rheumatologic follow-up, 80% of patients changed therapies: cDMARDs were added in 43.7% patients while bDMARDs were started in 40%. Systemic steroids were added in the remaining 16.3% of patients. At the latest follow-up (follow-up-time 33±28 months), 86% of patients had achieved remission of NIU while 14% had recurrence of active uveitis.ConclusionFindings from this population-based study describe a representative estimate of extent and patterns of NIU in an Italian adult cohort from a single Rheumatologic Clinic and document that Rheumatologic referral might dramatically impact treatment strategies in NIU.References[1]Hysa, E., et al. (2021). Immunopathophysiology and clinical impact of uveitis in inflammatory rheumatic diseases: An update. European journal of clinical investigation, 51(8), e13572. doi.org/10.1111/eci.13572.[2]Rosenbaum, J. T., et al. (2019). New observations and emerging ideas in diagnosis and management of non-infectious uveitis: A review. Seminars in arthritis and rheumatism, 49(3), 438–445. doi: 10.1016/j.semarthrit.2019.06.004.Disclosure of InterestsNone declared

BackgroundPsoriasis (PSO) is a systemic immune-mediated disorder, characterized by inflammation skin and joint manifestations: it is known that up to 30% of PsO patients develop PsA.PsO and PsA share common etiopathogenetic pathways, as IL-23/IL-17 axis. In Italy, Guselkumab (GUS), a selective IL-23 inhibitor, was approved in 2018 for the treatment of PsO, and recently it was also approved for PsA therapy. Today, there are few “real-world” studies regarding the use of GUS in PsA patients.ObjectivesA multicenter Italian study group of dermatologists and rheumatologists aimed at evaluate GUS efficacy and safety in patients with concomitant PsO and PsA in real word setting on both skin and joint domains.MethodsAn observational retrospective, multicentric study was performed in 69 PsO patients with a confirmed diagnosis of moderate to severe PsA. PASI and DLQI were used for the evaluation of skin response, and the number swollen/tender joints, presence or absence of dactylitis or enthesitis or axial involvement and painVAS were evaluated for the articular and periarticular efficacy. These analyses were performed at baseline T0 (beginning of the therapy), T1 (12 weeks) and T2 (24 weeks).Results38/69 patients (55,1%) presented oligoarthritis, 31/69 (44,9%) showed polyarthritis, none of the patients had enthesitis or axial involvement. Moreover, co-morbidities were diagnosed: hypertension (52,2%), hypercholesterolemia (34,8%), Hypertriglyceridemia (29%), diabetes (24,6%), obesity (23,2%), HIV-positive (20,3%), psychiatric disorders (17,4%), cardiopathies (15,9%), inflammatory bowel disease (7,3%), Latent Tubercolosis (4,4%), Chronic B-Hepatitis (2,9%), Chronic C-Hepatitis (1,5%). In all these patients, skin and joint responses were evaluated at week 12 and week 24.Concerning skin efficacy, PASI 90 was achieved at week 24. Concerning Joint response: painVAS progressively improved till T2, tender joint count decreased in patients with oligo and polyarthritis at T1 and maintained at T2, while swollen joint count decreased in polyarthritis patients at T1 and maintained at T2. In oligo-arthritis patients, this parameter was not improved. The number of dactylitis did not decrease during the period of study (see Table 1). No safety concerns were reported in this population.ConclusionEfficacy and safety of GUS was confirmed in this study group of PsO patients with concomitant PsA and several comorbidities in a real-life setting.Disclosure of InterestsNone declared

The efficacy of tazarotene 0.1% gel in a 30-year-old woman with type I lamellar ichthyosis is reported. The drug was applied to 15% of the total body surface area as follows: once daily for 2 weeks, three times a week for further 2 weeks, followed by a once weekly maintenance application. During the first week of treatment there was partial improvement obtained and in the next 14 days further reduction of scaling within the tazarotene-treated areas was observed. After 4 months of maintenance application, there was a marked overall improvement in the treated areas. Side-effects consisted only of mild pruritus, slight burning and irritation. In essence, the therapeutic benefit obtained was comparable with that of systemic retinoids but without the adverse systemic side-effects. As noted in other reports, tazarotene 0.1% gel seems to be a valuable and safe therapeutic option for this severe genodermatosis.