Explore the vast range of titles available.

Large Vessel Vasculitis (LVV) are vasculitides affecting large arteries, including aorta and its major branches. LVV can cause systemic and vascular inflammation that could result in long term complications such as artery stenosis, aneurysm formation and artery dissection. LVV include giant cell arteritis (GCA) and Takayasu. CGA typycally affects people aged over 50 and involves superficial temporal arteries. Meanwhile its cranial manifestations are well recognized and can be easily diagnosed by vascular ultrasound, it has been appreciated that GCA can extend beyond the superficial cranial arteries in up to 80% of patients or even spare the temporale arteries, and ultrasound is of limited diagnostic value in these cases. The usefulness of [18F]FDG PET/CT in supporting the diagnosis of LVV is well known, particularly in patients with less specific manifestations such as fever or unexplained weight loss. However, the role of [18F]FDG PET/CT in monitoring the course of the disease and its complications is less well established. To investigate the findings of repeated [18F]FDG PET/CT scan in a cohort of patients with LVV in clinical and biochemical remission. We have evaluated 30 pts (female 67%, age 75±9) with a diagnosis of LVV based on clinical features and serological tests (T0). The diagnosis was also supported by a positive [18F]FDG PET/CT scan (arterial uptake equal or superior to the liver at the aorta or at least one of its major branches). 16 patients (53%) had also GCA confirmed by positive ultrasound of the temporal arteries. All patients were started on Prednisolone (PRED) 1 mg/kg according to standard guidelines, and some were subsequently started on Tocilizumab (TCZ) due to either relapse on steroid tapering or contraindications to long term use of steroids. After at least 6 months of effective treatment and while on clinical and biological controlled disease (T1), patients were reassessed clinically and biochemically; 14 pts aldo had a repeated [18F]FDG PET/CT scan. We have used a binary grading to score the second scan: 1. negative (improved compared to first study) or 2. positive (not improved compared to first study), where improvement has been defined as no uptake or uptake inferior to the liver in the same areas that were highly metabolically active at previous study, and no new sites of vascular uptake detected. Images have been assessed by a nuclear medicine doctor and reviewed by 2 rheumatologists with recognised experience in the field of vasculitides, with excellent interobserver agreement. Inflammatory markers significantly dropped between the 2 timepoints (ESR 48±28 vs 6±4 and CRP 5.9±5.4 vs 0.4±1.2, p=0.0004). Among the 14 patients that had a repeated scan, 9 (65%) showed a negative and 5 (35%) a still positive scan despite being in clinical and biochemical remission. The number of metabolically active sites was reduced with a statistically significant difference between the 2 groups (8±4 vs 3±3, p=0.02). Of the 5 pts that had a positive scan, only 1 (20%) had a positive ultrasound of the temporal arteries at the time of the diagnosis. Our study revealed persistent vascular uptake on repeated [18F]FDG PET/CT scan in over 1/3 of LVV patients with clinical and biological controlled disease. The significance of this residual uptake and its prognostic value is currently unknown. Therefore the routine use of [18F]FDG PET/CT alone in monitoring disease activity and treatment response may be not appropriate and therapy should not be modified based on scan results only. Further research is needed in order to place the correct use of imaging in the long term monitoring of LVV. [1]Dejaco C, et al. Ann Rheum Dis 2018;77:636–643. doi:10.1136/annrheumdis-2017-212649 [2]Duftner C, et al. RMD Open 2018;4:e000612. doi:10.1136/rmdopen-2017-000612 [3]van der Geest KSM et al. Eur J Nucl Med Mol Imaging 2021; 48:3886–3902 NIL. None Declared.

Background:Reproductive health is growing attention is all medical fields. As most rheumatic diseases are diagnosed at young age both in men and women, reproductive health is to be considered for a comprehensive management of our patients.Objectives:To assess whether reproductive health counselling is a valid choice for patients with rheumatic diseases.Methods:In this observational study, a survey about reproductive health (sexuality, contraception, fertility, pregnancy, lactation, transmissibility) in relation to their disease has been administered to patients with immune-mediated diseases aged 18 to 50years, attending a Reproductive Health Counselling Clinic. Their need for information about reproductive health as part of a counselling carried out by dedicated rheumatologists was investigated. A survey about their experience with our clinic is analyzed in this study.Results:From January to September 2023, 271 patients completed the survey (Figure 1), of which 102 (37.6%) asked for counselling. Of these 102 patients, 24 (23.5%) wanted to explore contraception, 49 (48.0%) fertility, 79 (77.5%) transmissibility, 46 (45.1%) drugs used for their rheumatic disease and 41 (40.2%) sexuality. 68.3% of women wanted to address pregnancy and lactation, while 7 men wanted to address conceiving. 35 counselling have already been carried out (22 females, 13 males); 4 of these counselling were televisits. 15 were couple counselling. The topics that patients needed to address during counselling were sexuality (16, 45.7%), fertility (22, 60.0%), contraception (11, 31%), conceiving/pregnancy/lactation (20, 571%), drugs used for their rheumatic disease (18, 51.4%). 7 patients were referred to other specialists, such as gynecologist (4 women), urologist (1 man), sexologist (2 patients). 2 patients were referred to other specialists. 12 women were referred to our rheumatologic-gynecologic multidisciplinary clinic for broodiness. Most of the patients, as shown in Figure 1, reported an optimal satisfaction.Conclusion:Reproductive health counseling conducted by dedicated rheumatologists, in-person or remotely, has proven to be an effective method for conveying useful information and providing appropriate guidance to male and female patients. The level of patients’ satisfaction was found to be high, both in terms of the quality of the information received and the positive impact on reducing fear and stress in relation to these issues, suggesting to implement this tool to improve the management and the quality of life of patients living with rhematic diseases.REFERENCES:NIL.Table 1. Demographic characteristics of patients. CTD, connective tissue disease; RA, rheumatoid arthritis; SpA, spondyloarthropathies; b-DMARDs, biological disease-modifying antirheumatic drugs; cs-DMARDs, conventional synthetic disease-modifying antirheumatic drugs; ts-DMARDs, targeted synthetic disease-modifying antirheumatic drugs.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:As reproductive health is gathering attention in Rheumatology and all medical fields, the male perspective on the matter is often overlooked as not directly related to pregnancy and lactation.Objectives:To describe male perception of sexuality, fertility, contraception, conceiving related to their rheumatic disease, and their need to explore these aspects in a dedicated counselling session.Methods:In this cross-sectional observational study, a survey about reproductive health (sexuality, contraception, fertility, conceiving, pregnancy, lactation, transmissibility) in relation to their disease has been administered to consecutive patients with immune-mediated diseases aged 18 to 50years, attending a Reproductive Health Counselling Clinic. Their need for information about reproductive health as part of a counselling carried out by dedicated rheumatologists (on-site or by televisit) was investigated. In this study the male perspective was analysed.Results:From January to September 2023, 103 male patients completed the survey (Table 1). 10% of patients noted a changing in their sexuality after the diagnosis, 20% patients thought their rheumatic disease influences their sexuality and 3.9% believed their partner sexual behavior changed after the diagnosis. Regarding contraception, 37.9% of patients affirmed not to use condoms, while 18% had a female partner that used contraceptives, and most patients had never explored the contraception topic with their partner. 10% and 17% of patients reported their fertility may be influenced by their disease or the drugs taken for it, respectively. From our data, 60% were worried about transmissibility of their disease to children. 37% of patients were worried both for the effect that their disease or the drugs taken for it could have on conceiving, respectively. Figure 1 shows which aspects of reproductive health had already been addressed during any visit and which aspects patients needed to address. It is important to underline that 44% of patients had never addressed any reproductive health issue with any physician, while 39% had already discussed these topics with a rheumatologist, 4% with a urologist and 7% with their general practitioner. 37 patients requested counselling, of which 15 have already been carried out (13 on-site visits, 2 televisits). Most of the patients was satisfied by the counselling (80%) and would recommend it to other patients (86.7%). 1 patient has been referred to a Urologist to address infertility issues.Conclusion:Reproductive health is often overlooked especially in male patients as not directly related to pregnancy or lactation. As almost 50% of male patients had never addressed any of reproductive health related items before (sexuality, fertility, conceiving, contraception, drugs taken for the rheumatic disease), counselling, both in-person or remotely, has proved successful in answering patients’ questions or doubts.REFERENCES:NIL.Table 1.Demographic and clinical characteristics of patientsCTD, connective tissue diseases; PsA, psoriatic arthritis; RA, rheumatoid arthritis; SpA, spondyloarthropathies; b-DMARDs, biological disease-modifying antirheumatic drugs; cs-DMARDs, conventional synthetic disease-modifying antirheumatic drugs; ts-DMARDs, targeted synthetic disease-modifying antirheumatic drugs.Figure 1.Acknowledgements:NIL.Disclosure of Interests:None declared.

Summary Increased fragility has been described in humans with polycythemia vera (PV). Herein, we describe an osteoporotic phenotype associated with decreased osteoblast activity in a mouse model of PV and another mouse of polycythemia and elevated circulating erythropoietin (EPO). Our results are important for patients with PV or those treated with recombinant EPO (rEPO). Introduction PV and other myeloproliferative syndromes have been recently associated with an increased risk for fractures. However, the presence of osteoporosis in these patients has not been well documented. EPO, a hormone primarily known to stimulate erythropoiesis, has been shown recently to regulate bone homeostasis in mice. The aim of this study was to examine the bone phenotype of a mouse model of PV and compare it to that of animals with polycythemia caused by elevated circulating EPO. Methods Bone mass and remodeling were evaluated by micro-computed tomography and histomorphometry. The JAK2 V617F knock-in mouse, a model of human PV, manifests polycythemia and low circulating EPO levels. Results from this mouse were compared to wild type (wt) controls and the tg6 transgenic mouse that shows polycythemia caused by increased constitutive expression of EPO. Results Compared to wt, both JAK2 V617F and tg6 mice had a decrease in trabecular bone mass. Tg6 mice showed an additional modest decrease in cortical thickness and cortical bone volume per tissue volume ( P  < 0.01) suggesting a more severe bone phenotype than JAK2 V617F . Decreased osteoblast numbers and bone formation along with normal osteoclast numbers and activity were found in both mice. Conclusions This study indicates that PV is associated with low bone mass and decreased osteoblast activity in mice. Our results support future studies of osteoporosis in affected humans. Polycythemia caused by chronically elevated circulating EPO also results in bone loss, and implications on patients treated with rEPO should be evaluated.

Background:Behcet disease is a rare inflammatory disorder with the unique ability to affect vessels of any size. The disease could be associated to thrombosis in both the venous and arterial compartment, and often aneurysms. In particular, the presence of aneurysms of the pulmonary artery is rarely, if ever, seen in conditions other than Behcet. Cardiac involvement, albeit uncommon, is also described and associated to a severe prognosis. The treatment is based on immunosuppressants, meanwhile the use of anticoagulants -especially when aneurysms are present- is debated.Objectives:To describe a complex case of Behcet disease.Methods:We report the case of a 45 years old man of Chinese origin who presented to A&E with fever and acute dyspnea. Blood test revealed raised ESR and CRP and raised neutrophil count. Chest X rays showed bilateral opacities suggesting pneumonia. The patient did not improve over the course of antibiotics. Later on, he presented with an episode of hemoptysis and worsening dyspnea, so he was admitted to the Intensive Care Unit. CT showed bilateral pulmonary thromboembolism and aneurysm of the pulmonary artery. Echocardiogram and cardio-MRI revealed a large, mobile thrombus within the right atrium. Extensive work-up for infections and cancer was unrevealing. ANA, ENA and ANCA antibodies were negative. On the basis of a past medical history of recurrent oral ulcers and papulopustular skin lesions that patient admitted on questioning, a diagnosis of Behcet disease was suspected. In keeping with that, HLA-B51 turned out positive. The patient was promptly started on IV steroid pulses followed by Cyclophosphamide 1 gr IV monthly for six months, then on IV anti-TNF alpha Infliximab. He was also commenced on low molecular weight heparin (LMWH) and subsequently direct factor Xa inhibitor Apixaban.Results:The patient improved significantly with progressive regression of the pulmonary CT changes. He was discharged and able to get back to his daily life activities. After 2 years and a half of treatment, the aneurysm was stable and the intracardiac thrombus completely cleared.Conclusion:This case is of particular interest because of the concomitant presence of two rare vascular complications of Behcet disease-intracardiac thrombosis (<1-2%, less than 100 cases described worldwide) and pulmonary artery aneurysm (1-2%). Prompt introduction of immunosuppressant therapy was associated with a favorable outcome with no recurrence. We could speculate that, to some extent, the concomitant use of anticoagulants may have contributed to the complete resolution of the intracardiac thrombosis.Disclosure of Interests :MARIA DI CICCO: None declared, oscar massimiliano epis Consultant of: yes, Speakers bureau: yes, Cinzia Casu: None declared, Antonella Adinolfi: None declared, Luisa Alvaro: None declared, Valeria Campanella: None declared, Michel Chevallard: None declared, Marina Muscarà: None declared, Mariaeva Romano: None declared, Emanuela Schito: None declared, Nicola Ughi: None declared, Elisa Verduci: None declared, Davide Antonio Filippini: None declared

Background Thrombotic events are formal clinical criteria for the diagnosis of Antiphospholipid Syndrome (APS). By now there are no evidences if there is an antibody profile or a specific risk factor that can predict onset and/or recurrences of thrombosis in APS patients. Objectives Retrospective analysis of antibody profile and cardiovascular risk factors in patients with Primary Antiphospholipids Syndrome (PAPS) and thrombotic events. Correlation of these factors with type of thrombotic event (arterial-A, venous-V) and numbers of events (singular-S or recurrent-R). Methods The study included 96 PAPS patients (25 male, 71 female). Patients were divided into subgroups:-type of thrombotic event (A or V); -number of episodes (S or R). Mean age at first thrombotic event was 36.7 years (range: 12.6-68.9 yrs); mean age at first recurrent event was 40.8 yrs (range: 19.7-70.8 yrs); medium time of follow-up was 134 months(range: 8.2-427). Results 61% of patients begin with a V event, while 38% with an A event.V events occurred in patients with a mean age significantly lower compared with patients with A events (median value:2 9.7yrs vs 38.4yrs, p<0.01). Regarding each cardiovascular risk factor considered, hypertension and cardiac disorders seemed to be the most frequent in patients with A vs V thrombosis (15.3% vs 54.1%, p<0.0001; 10.2% vs 67.6%, p<0.0001). In 31 patients(32.3%) we identified the trigger for the first thrombotic event, represented in most cases by the concomitant use of estroprogestinic treatment and by pregnancy or post-partum period. During the follow-up visits we reported 58 R in 38 patients(39%). Compared with the first event, 20 R occurred in the same area(5 A and 15 V)while others 18 R occurred in different areas than the previous. In 23 patients only one R was recorded, in 10 patients two R and in 5 patients three or more R. In younger patients the recurrence of thrombotic events was significantly associated to the triple positivity for antiphospholipid antibodies(aPL)(76.2% in patients<45 yrs-old vs 25.6% in patients>45 yrs-old, p=0.01). In all patients with R the most frequent trigger was the interruption of the therapy or an inadequate control of the anticoagulation therapy. In particular 61% of these patients did not take for various reasons any therapy and in 52.2% of cases that therapy was interrupted not more than 10 days. Conclusions In our cohort, R of thrombosis occurred in about 40% of patients during a mean follow-up period of 10 yrs. The inadequate adherence to treatment, as expected, is significantly associated to R in over 50% of cases. In younger patients triple positivity for aPL is related to R. This finding underlines that it’s mandatory to consider the antibody profile during the risk stratification of our patients, together with all cardiovascular risk factors and other possible triggers like pregnancy/post-partum period or estroprogestinic treatment. References Myakis S, et al. International consensus statement on an update of the classification criteria for definite Antiphospholipid Syndrome.J Thromb Haemost2006 feb;4:295-306. Ruiz-Irastorza G, et al. Evidence-based reccomandations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients:report of a task force at the 13th International Congress on Antiphospholipid antibodies. Lupus2011;20(2):206-218. Disclosure of Interest None Declared

BackgroundThere is evidence that autoimmunity, namely RF and ACPA antibodies, may influence disease activities and impact the clinical outcomes in RA.ObjectivesThere is evidence that autoimmunity, namely RF and ACPA antibodies, may influence disease activities and impact the clinical outcomes in RA.MethodsWe analysed longitudinal data of consecutive RA patients from the Italian registry GISEA, starting a treatment with golimumab (GOL) and tested for rheumatoid factor (RF) and anti-citrullinated protein antibodies (ACPA). Demographic and disease related characteristics were collected at baseline, 6 months, 12, and 24 months or at last observation visit. Primary endpoint was the persistence on GOL in RF/ACPA +ve and RF/ACPA patients. Secondary endpoint was the search of baseline predictors of drug survival and clinical outcomes n the two RA subsets. Drug survival was evaluated by Kaplan-Meier life table analysis. Estimates hazard ratios (HRs, 95% confidence intervals (CI)) of drug discontinuation or achievement of low-disease adjusted for patient’s demographics, disease characteristics and prior biologic treatments were computed by Cox-regression stepwise backward models.Results345 patients had data on RA and ACP testing and were included in this analysis. No significant difference in terms of age, BMI, disease activity, co-therapy with glucocorticoids or methotrexate (MTX) was detected between RF/ACPA+ve and RF/ACPA-ve patients, but the former had significantly higher disease duration (10.6±vs 8.2±6 years) and frequencies of comorbidities (60.6% vs 44.2%). The 2 years global drug retention was 64.5%, and it was almost identical in RF/ACPA+ve 64.2% and in RF/ACPA-ve 65% RA patients. Drug survival was not influenced by the gender or cause of discontinuation (adverse or inefficacy). To note, in 31% of the patients GOL was not associated to MTX.The only predictor of drug discontinuation was the lack of MTX at baseline (HR 1.62, 95 CI 1.07–2.46, p=0.02), and the GOL-naïve status (HR 0.62, 95 CI 0.39–0.99, p=0.04). At two years, 44.4% achieved the state of low-disease activity (DAS28 <3.2) with no difference between RF/ACPA+ve (45.4%) and RF/ACPA-ve (42.0%) patients, and none baseline factor correlating with low disease activity. No safety issues were raised during the study.ConclusionsIn this study, we demonstrated that RF/ACPA positivity does not negatively impact on drug survival on golimumab. This may aid rheumatologists in their clinical decisions.Disclosure of InterestNone declared

According to the classification criteria of antiphospholipid syndrome, lupus anticoagulant, anticardiolipin and anti-β2 glycoprotein I antibody assays are independent risk factors for the occurrence of vascular thrombosis and pregnancy loss. However, it is generally accepted that patients carrying multiple positivity have more a severe disease and higher recurrence rate despite treatment. On the other hand, the diagnostic value of a positive result in one only assay is more controversial, particularly in the presence of clinical manifestations such as deep vein thrombosis or early miscarriages, which are rather common in the general population. In this review we speculate on current and future strategies to interpret different antiphospholipid antibody profiles in the clinical practice. Lupus (2010) 19, 432—435.

Background The role of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (anti-CCP) in the response to treatment for rheumatoid arthritis (RA) is not fully understood. Studies investigating changes in the levels of RF in response to synthetic and biological DMARDs have not been able to confirm a definitive relationship between decreased RF subtypes and clinical response, while studies investigating changes in anti-CCP levels have yielded conflicting results. In addition, to our knowledge the correlation between autoantibodies and tocilizumab treatment has been poorly investigated. Objectives This study investigates the relationship between the presence and levels of RF and anti-CCP and clinical response to tocilizumab in patients with RA. Methods This was an observational longitudinal study in 27 patients with active, long-standing RA despite previous treatment with >2 DMARDs and/or steroids. Patients were treated with tocilizumab 8 mg/kg every 4 weeks. All patients were assessed using approved clinical scales (VES, PCR, HAQ, DAS28-VES, DAS28-PCR, CDAI, and SDAI). IgM-, IgA- and IgG-RFs and anti-CCP antibodies were assessed using ELISA at baseline, 3 months (T1), 6 months (T2), and 12 months (T3). Results All patients showed significant and sustained clinical response to tocilizmuab treatment. All clinical scales with the exception of HAQ significantly decreased. There was a significant correlation (p=0.03) between anti-CCP and SDAI changes from baseline at T1 and T2. Likewise there were no significant correlations between antibody count at T0 and changes in the DAS-28 VES at T1 and at T2. No significant relationship between clinical scales and antibody levels RF-IgG, IgA, IgM and anti-CCP levels were observed. Conclusions Tocilizumab is effective in treating the clinical symptoms of RA, and the efficacy of this molecule was not correlated with either RF or anti-CCP levels. There is now a growing body of evidence suggesting that markers associated with clinical response may not be the same biomarkers that predict risk of further joint damage References MIKULS TR, O’DELL JR, STONER JA, ET AL: Association of rheumatoid arthritis treatment response and disease duration with declines in serum levels of IgM rheumatoid factor and anti-cyclic citrullinated peptide antibody. Arthritis Rheum 2004; 50: 3776–82. KARSDAL MA, WOODWORTH T, HENRIKSEN K, ET AL: Biochemical markers of ongoing joint damage in rheumatoid arthritis--current and future applications, limitations and opportunities. Arthritis Res Ther 2011; 13: 215 TAKEUCHI T, TANAKA Y, AMANO K, ET AL: Clinical, radiographic and functional effectiveness of tocilizumab for rheumatoid arthritis patients-REACTION 52-week study. Rheumatology 2011; 50: 1908–15. Disclosure of Interest None Declared

Background Neuropsychiatric (NP) involvement in systemic lupus erythematosus (SLE) includes a wide variety of neurologic and psychiatric manifestations whose attribution to the underlying disease is a clinical challenge. Objectives To determine the sensitivity and specificity of an attribution model (AM) applied to a large multicenter series of NP events. Methods All NP events satisfying the 1999 ACR case definition criteria occurred in a multicenter cohort of SLE patients (pts), in a timeframe of 5 years, were retrospectively analyzed. All NP events were tested by applying an arithmetical AM, firstly developed in a cohort of pts recruited in Ferrara, which included a set of 4 items selected on the basis of a literature review and by a Delphi consensus methodology during the XLVII Congress of the Italian Society of Rheumatology. The items included in the AM were the following: time of onset of NP event respect to the SLE clinical onset (before -0.5; after +0.5, concomitant +1); “minor” or not specific NP events as defined by Ainiala et al. (yes = -1; not = +0.5); opposing factors or “associations” as defined by ACR glossary (none =0; 1 = -0.5; >1 = -1); other favoring factors such as age of onset, absence of familial history for epilepsy or psychiatric disorders, abnormal serology, neuroimaging abnormalities, response to treatment, high disease activity at the time of the event (none =0, 1 =+0.5, >1=+1). According to the final score each NP event was then classified as related (≥+1), uncertain (-0.5 to +0.5) or SLE-unrelated (≤-1). The performance of the AM was estimated by its application to an external cohort of pts by the ROC curves analysis, assuming the “clinical judgment” performed by each attending team as the referral gold standard. Results 211 eligible pts were included, 91% F e 9% M, with a mean age of 33.5 yrs for a total of 430 events evaluated. Applying the AM 3 classes of values were generated: 179 events were classified as SLE related, 29 as SLE-unrelated and the remaining as uncertain events. After exclusion of uncertain events, the result obtained by the ROC curves analysis comparing the results of the AM with the clinical judgment, yielded a sensitivity of 62% and specificity of 93% when all the NP events were considered; when the analysis was restricted to first NP event only, the sensitivity raised up to 72% and specificity was 90%. Conclusions The overall performance of a new arithmetical AM was satisfactory in terms of sensitivity and specificity and allowed a proper placement in three-quarter of the NP events deemed as related and unrelated to SLE. This model can be regarded as an easy to use tool which allows a standardized approach to the complex sphere of NP involvement in SLE. Disclosure of Interest None Declared