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The objective of this study was to evaluate the efficacy of the endothelin receptor antagonist, bosentan, in patients with Raynaud's phenomenon secondary to systemic sclerosis never treated with prostanoids and without digital ulcers. The study design is a preliminary, prospective open label trial. The patients recruited took one 62.5 mg dose of bosentan twice daily for 4 weeks, followed by 125 mg twice daily for 24 weeks. Of the 10 patients recruited, all completed the study. The reduction in Raynaud's phenomenon attacks at week 24 from the baseline was statistically significant (Δ-1.3, P=0.0126). The Raynaud's condition score showed a statistically significant improvement (Δ-1.4, P=0.0279), as did the visual analog pain scale (Δ-1.5, P=0.0016) at the 24th week. Bosentan appears to be effective and may be a valid alternative for the treatment of severe secondary Raynaud's phenomenon for patients where prostanoids therapy is contraindicated or refused.

Giant cell arteritis (GCA) is the most common primary systemic vasculitis in western countries with the highest incidence among persons 70–79 years of age. Treatment has been with glucocorticoids (GCs) alone for many decades but recently Tocilizumab (TCZ) has demonstrated efficacy in reducing GC dose and flare rates in patients with GCA. Therefore, both early diagnosis and regular monitoring are necessary for the correct management of GCA. The COVID-19 pandemic has led to decisions by the governments of the countries involved, aimed above all at reducing the contagion. This has also led to reductions in health activities, limiting them to those of urgency by reducing or canceling checkups involving the risk of a time gap which for the GCA meant the interruption of clinical monitoring and therapeutic adjustment. At the same time, the pandemic situation has stimulated remote monitoring activities, through telephone contacts or video calls carried out by the rheumatologist. EULAR identified a minimal data set aimed at research and for clinical use, which includes the main clinical and instrumental data to be taken into consideration in monitoring the patient. For many data a clinical examination is not necessary but an interview is sufficient. We activated the TELEMACOV protocol (TELEmedicine and Management of the patient affected by giant cell arteritis during the COVid-19 pandemic) monitoring the follow-up of patients affected by GCA through telemedicine tools in order to maintain an effective and risk-free follow-up in a pathology with a high risk of relapse. The purpose of the study is to evaluate the effectiveness of telemedicine in the follow-up of the patient with GCA. We evaluated patients (pts) with a clinical diagnosis of GCA (received in previous periods) who were admitted to the our Rheumatology Unit. They were monitored monthly by telephone from 9 March to 9 June 2020 (during lockdown). All patients were asked questions divided according to the sub-groups listed below: - Onset of new symptoms or their recurrence - Exams carried out - Current therapy - Satisfaction of telephone call We performed 148 remote monitoring visits in 37 pts. The cohort was mainly composed by female (77,8%) and had a mean age of 71,85 ± 9,25 years. They were affected by GCA, with a mean duration of 5,3±2,3 months. The characteristics of these pts and the course of the disease are reported in Table 1. Pts treated with TCZ reduced their GC dose more than patients treated with GC alone (p: 0.032). Only one patient (treated with GC alone) had an ocular flare with the need to increase the dosage of the GC with good response and rapid improvement. Furthermore, all patients considered this type of monitoring very satisfactory according to the Likert scale (1-5) with mean 4,4±0,2. Our study has shown how telemedicine can be well used in pts with GCA as a possible alternative, for a limited period, to traditional visits, especially in a fragile population such as the elderly and more exposed to the risk of SARS-COV2 infection. [1]Ehlers L et al. Ann Rheum Dis 2019 Sep;78(9):1160-1166 [2]Wagner E et al. Prim Care. 2012 Jun;39(2):241-59. None declared Table 1Disease course and remote monitoringParametersBaseline (at diagnosis)Pre-lock downFirst InterviewSecond InterviewPatients treated with only GC (19 pts)MeanSDMeanSDMeanSDMeanSDESR74,0823,9513,028,212,756,429,846,63CRP mg/l47,442,21,011,11,552,291,562,24Hgb mg/dl11,62,613,22,2412,753,1913,582,12PGA (1-10)7,552,052,711,792,241,592,151,04EGA (1-10)6,991,852,191,421,971,131,430,97GC mg52,7218,310,16,957,64,516,673,1Patients treated with GC and TCZ (18 pt)ESR74,5636,5811,33,859,894,713,374,4CRP mg/l47,841,71,130,651,580,851,620,73Hgb mg/dl11,363,8812,984,9112,434,313,255,15PGA (1-10)7,143,112,472,151,890,41,930,72EGA (1-10)6,932,720,950,730,680,430,410,72GC mg51,7618,858,044,385,883,924,164,32PGA: Patient Global Assessment EGA: Evaluator Global Assessment;

Systemic Sclerosis (SSc) is an autoimmune rheumatic disease characterized by excessive production and accumulation of collagen in the skin and internal organs and by injuries to small arteries. Impairment of the musculoskeletal system is one of the main causes of disability in SSc, indeed, about 90% of these patients have a loss of hand function. To date, the degree of skin involvement is evaluated through a semi-quantitative method called Rodnan Skin Score (RSS) or Modified-RSS (MRSS). However, MRSS is a method that has limitations related to the operator and his experience and does not provide information on joint mobility. Arduino® is an open source integrated online platform based on easy to use hardware and software. It is a system for creating interactive projects by inserting a special configuration code, using the Arduino® development environment. Through this platform it is possible to create electronic devices with specific purposes to lead the possibility of integrating different kits (eg types of sensors) in relation to the object of study. We have therefore created an electronic instrument (Sclerodermic Hand Sensor - SHS) independent operator and easily reproducible in order to measure the degree of mobility (flexion) of the hand in patients with SSc (Fig.1). The aim was to evaluate whether the SHS was able to highlight significant differences between patients with SSc and healthy patients. We recruited 20 female patients with SSc according to ACR criteria with a mean age of 50.8 ± 15.5 years and 20 healthy (HC) patients with a mean age of 44.3 ± 10.8 years (Tab.1), in order to test the effectiveness and sensitivity of the SHS tool. The results showed a significant difference between the two groups of patients (SSc vs HC) independent of the measurement method used as expected (Goniometer SSc / HC: Δ45.80 ° p: 0.003 SHS SSc / HC: Δ65.17 ° p: 0.002, Fig.1c), however the device created with Arduino® proved to be more sensitive than the goniometric measurement in detecting the degree of joint flexion (p: 0.002). The flexion sensor, indeed, unlike the goniometer, evaluates the simultaneous articular excursion of the entire finger (MCF, IFP and IFD) and not just one segment (Fig.1). This technology application, thanks to the creation of dedicated electronic devices, allows the physicians to be supported in clinical practice with independent operating tools. The tool we propose could be a valid support in accurately assessing the joint and indirectly skin involvement of sclerodactyly in this type of patient, especially in the context of a clinical trial to evaluate the efficacy of a treatment. Further studies are needed to compare with other methods to assess hand disability in SSc such as the use of HAMIS (Hand Mobility in Scleroderma) test. [1]Sandqvist G et al. J Rheumatol. 2016 Jul;43(7):1356-62. [2]https://www.arduino.cc. [Display omitted] None declared Table 1SSc Patients CharacteristicsChararcteristicsSScPatients (n°, subset D=Diffuse; L=Limited)20 (9D/11L)Age, mean ± SD years50.8 ± 15.5Duration of Raynaud's Phenomenon (mean ± SD years)12.8 ± 4.4Duration of SSc (mead ± SD years)8.4 ± 3.6MRSS (mean ± SD years)15.9 ± 5.3

Upadacitinib (UPA), a JAK inhibitor engineered for a greater selectivity towards JAK1, has demonstrated a favourable benefit-to-risk profile in rheumatoid arthritis (RA) patients with an inadequate response to cDMARDs and biologic agents in randomized clinical trials. However, real-life data on its efficacy and safety are limited. We herein provide the first prospective multicentre experience on UPA efficacy and safety profile in RA in a real-life context, focusing on clinimetric and ultrasonographic (US) data. The primary aim of the study was to assess changes in clinimetric and US scores between different time point observations (baseline, 1 month, 3 months and 6 months). Secondary aims were to: (i) estimate the impact of biologic line of treatment and of concomitant therapies on response to therapy; (ii) explore changes in laboratory parameters; (iii) find potential predictive factors associated with response to therapy. Medical records of consecutive RA patients treated with UPA and referred to three Italian tertiary Centers were prospectively reviewed. Adult patients meeting ACR/EULAR classification criteria for RA were enrolled. Our real-life experience confirms the efficacy in terms of clinical and US improvement as well as displaying a good safety profile. The combination therapy with a conventional immunosuppressants predicts a significantly lower clinical and US improvement, likely reflecting a more severe and aggressive course worthy of ad-hoc and more in-depth investigation. An improvement in the SDAI and DAS28CRP was observed, already from the first month of therapy and has been maintained over time. In conclusion, these results demonstrated the short term efficacy of Upadacitinib 15 mg for up to 6 months and providing a prompt improvement of PROs. Even if further studies are needed to clarify those results, these novel findings may provide new insight for the management of UPA treatment in clinical practice. Our real-life experience confirms the efficacy in terms of clinical and US improvement as well as displaying a good safety profile. The combination therapy with a conventional immunosuppressants predicts a significantly lower clinical and US improvement, likely reflecting a more severe and aggressive course worthy of ad-hoc and more in-depth investigation. An improvement in the SDAI and DAS28CRP was observed, already from the first month of therapy and has been maintained over time. In conclusion, these results demonstrated the short term efficacy of Upadacitinib 15 mg for up to 6 months and providing a prompt improvement of PROs. Even if further studies are needed to clarify those results, these novel findings may provide new insight for the management of UPA treatment in clinical practice. NIL. NIL. None Declared. [Display omitted] Table 1Changes in clinimetric and ultrasonographic scores throughout the study period and significance levels (p values) for single comparisons between different observations in time.EndpointOverall significanceT0 vs T1T0 vs T3T0 vs T6T1 vs T3T1 vs T6T3 vs T6Synovitis grade< 0.0010.8040.090<0.001< 0.001< 0.001< 0.001PD signal grading0.0290.9140.3200.0260.0290.0090.066Tenosinovitis grading0.0120.9680.3700,0310,0180,0090,059DAS28-CRP0.0050.014< 0.001< 0.0010.2200.0150.035SDAI index< 0.0010.003< 0.001< 0.001< 0.001< 0.001< 0.001List of abbreviation: DAS28-CRP Disease Activity Score 28 – C-reactive protein, PD power Doppler, SDAI Simplified Clinical Activity Index, T0 baseline, T1 1 month evaluation, T3 3 month evaluation, T6 6 month evaluation

BackgroundSynovitis in Rheumatoid Arthritis (RA) is a phenomenon related to the development of erosions and progressive structural damage; early synovitis improvements are successfully associated with long-term clinical and structural outcomes.ObjectivesThe aim of this study was to evaluated the efficacy of abatacept in two cohort of patients treated with Abatacept as the first and second or third line of treatment.MethodsWe evaluated patients affected by RA (according to ACR 2010 criteria) and were divided into two groups:Group A: patients with moderate or severe active RA, non-responders to Metothrexate (MTX), bDMARDs naïve, treated with Abatacept 125 mg/wk;Group B: patients with moderate or severe active RA, non-MTX and anti-TNF responders, treated with Abatacept 125 mg/wk;The concomitant treatment with MTX was maintained unchanged in those patients who were taking it at stable doses before the start of the study (10–15 mg/week for ≥28 days); concomitant therapies such as low-dose systemic CS (prednisone ≤7.5 mg/day) and NSAIDs have been maintained for at least 4 weeks if stable. The activity of RA was calculated with the DAS28-CRP according to the clinical practice protocol (week 0,4,12,24). The Ultrasound (US) evaluation of the synovitis was done according to the Omeract criteria (Grey Scale and PDUS score: 0 to 3).ResultsWe recruited consecutively 34 patients with RA, 16 pts (male n=4; 25,00%) took Abatacept as the first line (Group A), and 18 pts (males n=5; 27.70%) took Abatacept as followed by another anti-TNF drugs (Group B). The mean age was 57.2±10.7 years (median 60, range 45–72); mean of DAS28 at baseline was 4.8±0.9 (median 4.7; range 3.9–5.6); mean duration of the disease was 15.3±5.7 years (median 10; range 3–22). Tab.1A constant improvement of the DAS28 score is shown in both groups examined until the end of the follow up, resulting respectively -Δ 2.0 for Group A (p<0.05) and -Δ 2.1 (p<0.05) for Group B. The total PDUS score decreased in both groups from week 4, with a mean change (95% CI) compared to baseline of −0.8 (range −1.4/–0.2) and progressive mean significant improvement until follow-up (Gr.A p<0.05; Gr.B p<0.05). No serious adverse events or infections were observed. Patients with ACPA positive showed a greater improvement trend compared to other patients in both groups (p: 0,068). Figure 1.Abstract AB0468 – Table 1Cohort of patients at baselineAbstract AB0468 – Figure 1PDUS evaluation of two cohorts with ACPA positiveConclusionsThe treatment with Abatacept, administered in the first or second or third line, has shown significant efficacy in reducing the synovial inflammation in patients with RA, monitored with clinical and ultrasonographic outcome. Moreover, we have not demonstrate statistically significant differences between two groups into the timing of improvement.Reference[1] D’Agostino T, et al. Early Response to Abatacept Plus MTX in MTX-IR RA Patients Using Power Doppler Ultrasonography: an open label Study.Ann Rheum Dis2012;71:18.Disclosure of InterestNone declared

BackgroundSystemic Sclerosis (SSc) is a chronic inflammatory autoimmune disease of the connective tissue characterized by vasculopathy and progressive fibrosis thickening of the skin and internal organs. Vascular damage is recognized as a diagnostic landmark in SSc, both in its limited and diffuse form. Micro- and macro-vascular damage have been involved in the pathology; early detection may be helpful in therapeutic planning and management. Subclinical cardiac damage can be detected with transthoracic echocardiography (TTE) while arterial stiffness can be measured by Pulse wave velocity (PWV). Both have been shown to have an independent predictive value in cardiovascular risk stratification. However it is not yet clear whether they are actually present and detectable in SSc patients, or if an association with a specific subtype exists (1).Objectivesto evaluate the presence of subclinical cardiac organ damage in patients with limited and diffuse SSc, in comparison with a cohort of healthy individuals.MethodsPatients with limited and diffuse SSc referred to the Rheumatology Unit in our Hospital, in according to 2013 ACR/EULAR Classification Criteria, were enrolled. The study was conducted in accordance with the Declaration of Helsinki and approved by our Ethics Committee. All patients underwent a complete transthoracic echocardiogram, and PWV evaluation, which were performed in agreement with the last International Guidelines (2). Past medical history and risk factors, clinical and biochemical data were collected. 23 healthy subjects, similar for demographic characteristics, were used as controls.Results41 patients (35 female,age 56.9±13.6 years), 21 with diffuse and 20 with limited SSc were recruited. Past medical history, cardiovascular risk factors, gender distribution and disease duration were similar in the two subgroups with no statistically differences. Patients with limited SSc were mean of 10 years older than patients with diffuse SSc. TEE parameters – left ventricular remodeling, atrial enlargement, diastolic disfunction - and hemodynamic indexes – PWV (6.5 [6;6.8] vs 7.0 [6.2;8.5], p=0.24) and Augmentation index (145.6±14.2 vs 149±20.6,p=0.52) did not show significant difference. Indeed, in the regression analysis, PWV was correlated to age (rho=0.60,p<0.001). When compared with healthy controls, again no significant difference emerged, considering echocardiographic and hemodynamic indexes.ConclusionsIn SSc, PWV seems to increase in according to age, with no additional impact of the specific pathology nor subtype. Vascular damage in the SSc population is likely not reflected in increased arterial stiffness or subclinical cardiac damage. Evaluation of SSc patients with PWV and TEE should not be routinely performed.ReferencesNgian GS, Sahhar J, Wicks IP, Van Doornum S. Arterial stiffness is increased in systemic sclerosis: A cross-sectional comparison with matched controls. Clin Exp Rheumatol 2014;32(6 Suppl 86):S-161–6.Lang RM, Badano LP, Mor-Avi V, Afilalo J, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: An update from the american society of echocardiography and the european association of cardiovascular imaging. J Am Soc Echocardiogr 2015, Jan;28(1):1–39.e14.Disclosure of InterestNone declared

BackgroundSjögren's Sydrome (SS) is a chronic multisystem autoimmune disease characterized by hypofunction of salivary and lacrimal glands. The result of the immune-mediate infiltration of the lacrimal gland is a severe dry eye disease. None of the commercially available artificial tear preparations and anti-inflammatory topical treatment has the properties of the human tears as Growth factors (GFs), that are necessary for maintenance of normal corneal epithelium. Platelet alfa granules are a major source of GFs and are rich in platelet derived growth factor (PDGF), which plays an important role in the maintenance of ocular surface and tear film stability (1).ObjectivesTo determine the efficacy and safety of autologous platelet lysate (APL) eye drops in patients with primary SS dry eye, refractory to standard therapy, in comparison to patients treated with artificial tears. We focused on the effect of APL on cornea morphology with the in vivo confocal microscopy (IVCM).MethodsWe included patients with a diagnosis of SS according to the classification criteria of the American-European Consensus (2), a dry eye severity level ≥2 (Dry Eye Severity Grading Scheme, Workshop 2007). Patients were assigned in two groups, according to the randomization criteria 1:1–Group A used autologous platelet lysate (BiomedDevice) QID for 90 days.–Group B used preservative-free artificial tears QID (Ialuronic Acid 0,2%) for 90 days.Ophthalmological assessments included: ocular surface disease index (OSDI), Schirmer test, fluoresceine score and break up time (BUT). Both Group A and Group B underwent IVCM; corneal basal epithelium, sub basal nerves, Langherans cells, anterior stroma activated keratocytes were evaluated. The study was conducted in accordance with the Declaration of Helsinki (1964) and approved by our Ethics Committee.Results43 patients (female:male =42:43) with mean age of 59,75 years (SD±13,10) affected by primary SS from 7,48 years (SD±5,56) were enrolled. The two groups (A=22 pts; B=21 pts) did not have significant differences at baseline in terms of disease (duration, autoantibodies) and treatment (steroids, DMARDs) characteristics. In group A, the mean of improvement of OSDI, fluorescein score and BUT values (Δ38.82; Δ1.0; Δ1.5 respectively) were higher than group B (Δ7.07; Δ0.0; Δ0.0 respectively) with statistical significance (p<0.05). The IVCM showed a significant increase in basal epithelium cells density, sub basal nerve plexus density and number and a decrease in Langerhans cells density (p<0.05) only in group A. No adverse events were observed in all patients treated.ConclusionsAPL has proved to be effective and safety in the treatment of SS dry eye and IVCM seems to be a useful tool to visualize cornea morphologic modifications. Further studies are required to evaluate the long-term effects and the timing of retreatment.ReferencesPezzotta S, Del Fante C, Scudeller L et al. Autologous platelet lysate for treatment of refractory ocular GVHD. Bone Marrow Transplantation 2012; 47, 1558–1563.Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002; 61:554–8.Disclosure of InterestNone declared

Background Systemic sclerosis (SSc) is a chronic autoimmune inflammatory pathogenic disease of the connective tissue, characterized by progressive fibrosis thickening of skin and internal organs. SSc is caused by the accumulation of collagen (gastrointestinal system, heart, lung, kidney, joints and muscles) and widespread of vascular disease damage. The first vascular event is Raynaud’s phenomenon(RP). Objectives In our study, we evaluated the efficacy of Aminaftone in the treatment of RP in 19 patients with SSc consecutively recruited and divided in two groups. Methods Group A:9 Patients that not tolerated endothelin receptor antagonist (ERA) due to side effects (eg increased> 3 times the liver enzymes, headache, edema of lower limbs), in treatment with ACE inhibitors, prostanoids and Aminaftone. Group B:10 Patients in treatment with ACE inhibitors, prostanoids and ERA All Patients were aged between 40 and 68 years (Group A 51.6±10.2;Group B 52.1±10.5), with SSc according to ACR criteria, presenting RP and DUs with an onset between 6 and 9 months.11 of the 19 patients had the limited form of SSc withpositive anticentromere antibody value and 8 of them had a diffuse form withpositive SCL-70 antibody value. The mid duration of SSc was 6.4 years with 10.8 years of average duration of onset of RP. The A group tooks three tablets of Aminaftone 75mg/day while the B group tooks two tablets of Bosentan 125mg/day. Follow-up was performed every 4 weeks for 6 months and each patient was given a diary to report on each check, which indicate: -Date of onset of Raynaud’s Phenomenon -Duration: minutes -Raynaud’s Condition Score(RCS): Limitation of daily activity on a scale of 1 to 10 (meaning 10 as a total inability to do the activity) -Pain VAS (1-10): 1 meaning the least pain and 10 as the maximum pain -Number of daily attacks -Ulcer onset (date) -Location ulcer/s Results The number of RP attacks in both groups of patients decreased from baseline to Week 24, obtaining statistically significance (ΔA-2,80 p-0,02;ΔB-3.10 p-0,01). The duration of the RP was decreased, but did not obtainstatistically significance (ΔA-10,90 ΔB-12,30). The RCS has shown a statistically significant improvement (ΔA-2,10 p-0,04;ΔB-2.3 p-0,03), as well as the pain VAS (ΔA-3,20 p-0,04;ΔB-3,5 p-0,02) at week 24. The number of DUs decreased in both group of patients and in most cases (especially in Group B), the lesions met complete resolution. Furthermore, in patients treated with Aminaftone, no extras DUs appeared. Our data showed a statistically significant improvement of baseline in the number of attacks of RP, VAS pain and RCS. Conclusions In conclusion, the use of Aminaftone for the treatment of RP in SSc patients, seems to have been effective, with good tolerability and no adverse events. Although the ERA (Bosentan) is more effective, we believe that, at least for those patients whose therapy with ERA is not tolerated, Aminaftone may be a viable alternative treatment for RP. Long term studies with a wider coverage must be performed to assess the effects of Aminaftone. References Kahaleh MB. Raynaud phenomenon and the vascular disease in scleroderma. Curr Opin Rheumatol 2004;16: 718–22. Arefiev Kait et al. Endothelin Receptor Antagonists for the Treatment of Raynaud’s Phenomenon and Digital Ulcers in Systemic Sclerosis. International Journal of Rheumatology Volume 2011, Article ID 201787,7 Disclosure of Interest None Declared