S7A:6 Baseline serum levels of baff or april are independent predictors of sledai response after 12 months of treatment with belimumab in patients with refractory systemic lupus erythematosus

Background Belimumab, a monoclonal antibody targeting BlyS (B lymphocyte stimulator), is used in refractory Systemic Lupus Erythematosus (SLE). Pivotal clinical trials showed that SLE patients with positive anti-dsDNA antibodies and reduced levels of C3 and/or C4 fractions were those more likely to be responders to treatment. Our study aims at exploring predictors of response to Belimumab in the post-marketing experience in consecutive SLE patients treated at a single centre. Methods Twenty-one patients received Belimumab intravenously at standard regimen (10 mg/kg at 0–15–30 days and then every 4 weeks). Anti-dsDNA were tested by Farr assay and C3/C4 levels by nephelometry. Biomarkers belonging to the TNF superfamily and related to B cell activity (BAFF, APRIL, sBCMA, sCD40L, sTACI, TWEAK) were tested by ELISA. All laboratory parameters were tested at baseline and every 6 months afterwards. SLE disease activity was assessed by SLEDAI-2K score. General linear modelling and correlation analysis were performed using SPSS. Results Enrolled patients were 2 males and 19 females with a median (25th-75th percentile) age of 38 (31–42) years. The disease duration at time of Belimumab start was 12 (8–19) years. The baseline SLEDAI score was 6 (4–9), the anti-dsDNA level was 26 (11–99) UI/ml, and their C3 and C4 level was 72 (56–86) and 9 (7–15) mg/dL, respectively. All the parameters of the TNF superfamily showed moderate/strong correlation (r values ranging from 0.543 and 0.989, p In contrast, C3, C4, anti-dsDNA, and SLEDAI were less likely to predict relative SLEDAI change at 12 month of Belimumab treatment (uncontrolled model: C3 p=0.410; C4 p=0.778; anti-dsDNA p=0.412) in this cohort of patients preselected for the treatment with Belimumab. Conclusions In this preselected 'real-life' cohort of refractory SLE patients fulfilling the requirements for Belimumab treatment baseline serum levels of BAFF or APRIL are independent predictors of response to treatment. Therefore, BAFF and APRIL could be useful for response estimation in patients qualifying for Belimumab treatment

Validation of the Italian version of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire

Objectives: The primary objective of this study was the translation and validation of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire into Italian, denoted as AAV-PRO_ita. The secondary objective was to evaluate the impact of ANCA-associated vasculitis (AAV) on quality of life (QoL) and work impairment in a large cohort of Italian patients. Methods: The study design took a prospective cohort study approach. First, the AAV-PRO was translated into Italian following the step guidelines for translations. The new AAV-PRO_ita questionnaire covered three disease domains: organ-specific and systemic symptoms and signs; physical function; and social and emotional impact. Second, Italian-speaking AAV patients were recruited from 17 Italian centres belonging to the Italian Vasculitis Study Group. Participants completed the AAV-PRO_ita questionnaire at three time points. Participants were also requested to complete the work productivity and activity impairment: general health questionnaire. Results: A total of 276 AAV patients (56.5% women) completed the questionnaires. The AAV-PRO_ita questionnaire demonstrated a good internal consistency and test–retest reliability. Female AAV patients scored higher (i.e. worse) in all thee domains, especially in the social and emotional impact domain (P < 0.001). Patients on glucocorticoid therapy (n 1⁄4 199) had higher scores in all domains, especially in the physical function domain (P < 0.001), compared with patients not on glucocorticoid therapy (n 1⁄4 77). Furthermore, patients who had at least one relapse of disease (n 1⁄4 114) had higher scores compared with those who had never had one (n 1⁄4 161) in any domain (P < 0.05). Finally, nearly 30% of the patients reported work impairment. Conclusion: The AAV-PRO_ita questionnaire is a new 29-item, disease-specific patient-reported outcome measuring tool that can be used in AAV research in the Italian language. Sex, glucocorticoids and relapsing disease showed the greatest impact on QoL

Early and Late Response and Glucocorticoid-Sparing Effect of Belimumab in Patients with Systemic Lupus Erythematosus with Joint and Skin Manifestations: Results from the Belimumab in Real Life Setting Study—Joint and Skin (BeRLiSS-JS)

Aim. To assess the efficacy of belimumab in joint and skin manifestations in a nationwide cohort of patients with SLE. Methods. All patients with skin and joint involvement enrolled in the BeRLiSS cohort were considered. Belimumab (intravenous, 10 mg/kg) effectiveness in joint and skin manifestations was assessed by DAS28 and CLASI, respectively. Attainment and predictors of DAS28 remission (&lt;2.6) and LDA (≥2.6, ≤3.2), CLASI = 0, 1, and improvement in DAS28 and CLASI indices ≥20%, ≥50%, and ≥70% were evaluated at 6, 12, 24, and 36 months. Results. DAS28 &lt; 2.6 was achieved by 46%, 57%, and 71% of patients at 6, 12, and 24 months, respectively. CLASI = 0 was achieved by 36%, 48%, and 62% of patients at 6, 12, and 24 months, respectively. Belimumab showed a glucocorticoid-sparing effect, being glucocorticoid-free at 8.5%, 15.4%, 25.6%, and 31.6% of patients at 6, 12, 24, and 36 months, respectively. Patients achieving DAS-LDA and CLASI-50 at 6 months had a higher probability of remission at 12 months compared with those who did not (p = 0.034 and p = 0.028, respectively). Conclusions. Belimumab led to clinical improvement in a significant proportion of patients with joint or skin involvement in a real-life setting and was associated with a glucocorticoid-sparing effect. A significant proportion of patients with a partial response at 6 months achieved remission later on during follow-up

AB0523 TAKAYASU ARTERITIS AND SACROILIITIS: A CASE-CONTROL STUDY

Background:A possible shared immunopathogenesis between Spondyloarthritis (SpA) and Takayasu Arteritis (TA) has been hypothesized and some clinical cases about SpA in TA patients have been reported (1). In clinical practice the diagnosis of sacroiliitis may be performed by X-ray, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI). In particular, CT findings of sacroiliitis include contour irregularities, joint space alterations, joint erosion, subcondral bone changes (osteoporosis or sclerosis), enthesitis, ankylosis. Meanwhile, TA patients performe routinely FDG-PET/CT scans for monitoring disease activity.Objectives:This study aims to understand if there is an increased incidence of sacroiliitis in TA patients.Methods:We collected retrospectively imaging data from FDG-PET/CT scans of 28 TA patients and 28 controls, matched for sex and age. Controls were selected among patients performing FDG-PET/CT in our Nuclear Medicine Unit, excluding patients with bone tumors, bone metastasis and thyroid cancers. The majority of controls were affected by lymphoma in complete remission. An expert rheumatologist read the CT-scans of sacroiliac joints.Results:No patients or controls demonstrated FDG-uptake in sacroiliac joints. In the control group we detected sacroiliac sclerosis in two cases: one due to degenerative changes, one to sacroiliitis (1/28, 4%). In the TA group four patients presented CT alterations suggestive for sacroiliitis: one bilateral erosion, one bilateral sclerosis, two monolateral sclerosis (4/28, 14%). One of these patients complained an inflammatory back pain.Conclusion:In our cohort of TA patients we demonstrated an increased prevalence of sacroiliitis, diagnosed by CT scan. Only one patient reported an inflammatory back pain, while three patients had radiological signs of previous sacroiliitis. These findings highlight the importance of looking for spondyloarthropathy in TA patients even if asymptomatic.References:[1]Guzel Esen S, Joint Bone Spine, 2019Disclosure of Interests:None declare

Can we use NOACS in APS?

Secondary thromboprophylaxis with low molecular heparin or vitamin K antagonists (VKAs) is recommended in patients with definite antiphospholipid syndrome (APS). Direct oral anticoagulant (DOACs) have been approved in different prothrombotic conditions and have numerous advantages compared to VKAs. Whether DOACs can be used for secondary prophylaxis in APS is an open question. Data from the TRAPS randomized controlled Trial, meta-analysis and case reports indicate that we should not treat patients with triple positive APS and/or arterial thrombi with routine doses of DOACS. On the other hand, data from the literature including, case series, meta- analysis and the RAPS trial indicate that there are low risk patients, such as patients who suffered from a venous but not an arterial thromboembolism and are LAC negative who may benefit from the treatment with DOACs. Prospective trials addressing these low risk patients are needed in order to consider DOAC treatment in such patients