Sloanrichards9152

Twelve (15%) patients relapsed during follow-up. The mean VDI score was 0.4 in the last visit. Nineteen (24.7%) patients had at least one damage item at the end of follow-up. Most frequent damage items were renal 11 (42%), ocular 4 (15%) and cardiovascular 4 (15%). CONCLUSIONS In this cohort the most frequent damage item was renal and was related to the disease itself. Damage score was higher in patients with more severe disease and treated more aggressively. Our results suggest that more effective treatment options are needed in a subgroup of patients with IgA vasculitis to prevent the damage related with the vasculitis, especially with more severe disease.OBJECTIVES To explore predictive factors including MMP-3 for achievement of low disease activity (LDA) at 52 weeks in bio-switch rheumatoid arthritis (RA) patients treated with abatacept, for whom obtaining a good clinical response can be difficult. METHODS Participants were 423 consecutive patients with RA treated with abatacept who were observed for longer than 52 weeks and registered in the TBCR, a Japanese multicentre registry system. Multivariate logistic regression analysis was used to study factors that predict the achievement of LDA at 52 weeks in bio-naïve (n=234) and bio-switch (n=189) groups. RESULTS ROC analysis revealed that MMP-3 improvement rates at 12 weeks in bio-switch patients had the highest AUC with a cut-off value of 20.0% for predicting LDA achievement at 52 weeks. Multivariate logistic regression analysis revealed that, in addition to DAS28-CRP at baseline, achieving 20% improvement in MMP-3 levels at 12 weeks was an independent predictive factor (adjusted OR 4.277, p=0.003) in the bio-switch group, whereas DAS28 was the only predictor in the bio-naïve group. Patients who achieved 20% improvement in MMP-3 levels at 12 weeks had significantly higher achievement rates of LDA at 52 weeks compared to those who did not achieve 20% improvement in the bio-switch group (60.0 vs. 33.3%, p=0.001). CONCLUSIONS Our findings suggest that improvement in MMP-3 levels is key to predicting the clinical efficacy of abatacept. Closer attention paid not only to major clinical indices, but also changes in MMP-3 levels, could improve our ability to optimise clinical results when treating bio-switch patients.OBJECTIVES To assess subclinical vascular features in patients with systemic sclerosis (SSc) via carotid ultrasound, and flow-mediated vasodilation (FMD), as measures of cardiovascular risk (CVR). METHODS Cross-sectional study over a cohort of 70 patients diagnosed with SSc (diffuse or limited forms), on whom a vascular study protocol was performed to assess angiodynamic parameters measured by FMD in brachial artery and carotid ultrasound lesions carotid intima-media thickness (CIMT) and carotid atheroma plaques (AP). Classical CVR factors were also assessed, as well as main features of SSc regarding skin and organic involvement, laboratory parameters, presence of autoantibodies and specific treatments. RESULTS 94% of patients were women with a mean age of 50.2± 12.5 years. 84% had endothelial dysfunction (ED), being severe in 49%, statistically associated with glucocorticoid (GC) treatment (OR=8.78; CI= 1.52-50.78; p= 0.015). CIMT was pathological in 39%, 23% had AP (none had significative haemodymanic stenosis). Serum vitamin D concentration (25(OH)D3) showed a protective effect on CIMT (OR= 0.94; CI= 0.89-0.99; p= 0.025). No differences between types of SSc were obtained; neither association between SSc features and classical CVR factors. CONCLUSIONS GC treatment has implications in CVR, despite in SSc GC doses administered are lower than in other autoimmune diseases (in our cohort even prednisone ≤10 mg daily was associated with ED). GC may be associated with an early vascular damage in these patients, which could lead to changes in FMD, ED and finally AP. On the other hand, optimum levels of 25(OH)D3 seemed to be beneficial against vascular damage.OBJECTIVES Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease that affects various organs of the body. The aim of this study is to elucidate the clinical characteristics of IgG4-RD with central nervous system (CNS) involvement. METHODS Among 589 patients with IgG4-RD in a prospective single-centre cohort study in Peking Union Medical College Hospital, 15 patients had CNS involvement. The clinical data including demographic features, symptoms, involved organs, laboratory findings, radiological results, pathology, treatment and outcome were analysed. RESULTS Seventeen patients, including nine men and six women, had IgG4-related neuropathy, with an average age of 49.8±12.3 years. IgG4 related hypophysitis was the most common manifestation, accounting for 40% (6/15) of cases, followed by hypertrophic cranial pachymeningitis (n=4), hypertrophic spinal pachymeningitis (n=2), intracranial mass (n=2), cavernous sinus and orbital disease (n=1). Most patients had multi-organ involvement, with the most common extra-CNS manifestations were Mikulicz disease (MD) and lymphadenitis in 5 (33.3%) cases. Serum IgG4 levels were elevated in 12/15(80%) patients and the median value was 438.5 (104, 2250)mg/dL. Fourteen cases underwent biopsy, of which tissue was taken directly from CNS lesions in 4 cases. All patients received treatment with glucocorticoids (GCs) combined with immunosuppressants, including cyclophosphamide, tacrolimus, mycophenolate mofetil, and tripterygium glycosides. Complete remission was achieved in 3/15 (20.0%) patients, and 11/15 cases (73.3%) achieved partial remission. CONCLUSIONS IgG4-related CNS involvement is a rare and distinct entity of IgG4-RD. Treatment with corticosteroids combined with immunosuppressive agents yielded favourable responses.OBJECTIVES We evaluated age at natural menopause and the prevalence of premature ovarian failure (POF) in a monocentric Caucasian cohort of patients with systemic lupus erythematosus (SLE). METHODS In this cross-sectional study, we enrolled women affected by SLE compared with healthy controls (HC) to investigate data about natural menopause (amenorrhoea for at least 12 months at ≥40 years) and POF (amenorrhoea for at least 12 months at less then 40 years). RESULTS We enrolled 196 SLE (median age 47.0 years, IQR 16.7; median disease duration 132 months, IQR 180) and 90 HC (median age 49.9 years, IQR 15.0). Ninety-four SLE (48.0%) and 26 HC (23.4%) were menopausal median age at onset was significantly lower in SLE than HC (47 years, IQR 8.0 vs. 50.5 years, IQR 4; p=0.0001). POF was registered in 17% of the SLE, and in none of the HC (p less then 0.0001). POF was significantly associated with anti-Sm (p=0.0004), anti-RNP (p=0.02), anti-cardiolipin (p=0.0008), lupus anticoagulant (p=0.0002), treatment with cyclophosphamide (p=0.0001), azathioprine (p=0.0001), mycophenolate mofetil (p=0.0001), cyclosporine A (p=0.007). CONCLUSIONS SLE patients develop menopause at a younger age; moreover, a higher POF frequency was observed in SLE patients in comparison with HC. POF is associated with specific SLE-related autoantibodies and the use of immunosuppressant drugs, in particular cyclophosphamide.OBJECTIVES Takayasu's arteritis (TAK) is characterised by inflammation and fibrosis in the aortas, but its pathogenesis remains unclear. The aim of the study is to demonstrate the role of cysteine-rich protein 61 (CYR61), a novel proinflammatory factor, in the inflammation and fibrosis of TAK vessels. Stenoparib clinical trial METHODS CYR61 expression in the aortic vessel was compared between TA tissues and healthy samples by immunohistochemistry staining. The effect of CYR61 on the proliferation, migration and activation of adventitial fibroblasts (AFs) in the IL-17-mediated inflammatory microenvironment was studied in vitro. RESULTS Here we found higher expression of CYR61 in the aortic adventitia in TAK patients than in healthy donors by immunohistochemistry staining. In vitro, recombinant human CYR61 (rhCYR61) significantly upregulated the proliferation of primary human aortic adventitial fibroblasts (AFs) and their expression of extracellular matrix (ECM) proteins such as collagen I, collagen III and fibronectin at the mRNA and pt in TAK.OBJECTIVES Subclinical atherosclerosis, defined as the presence of carotid plaques, is more frequently found in patients with axial spondyloarthritis (axSpA) than in healthy individuals. We sought to determine whether axSpA patients are more commonly reclassified into the very high cardiovascular risk category than controls after performing carotid ultrasound and if this can be linked to disease characteristics. METHODS 343 patients diagnosed with axSpA according to ASAS criteria and 177 controls were studied. Disease characteristics and Systematic Coronary Risk Evaluation (SCORE) were assessed in patients and controls. Presence of plaques and intima-media thickness (cIMT) was determined by carotid ultrasound. Multivariable regression analysis was performed to identify differences in the frequency of reclassification between patients and controls, as well as factors associated with reclassification in axSpA. RESULTS Carotid plaques (36% vs.25%, p=0.010) and higher cIMT (0.641± 0.121 vs. 0.602± 0.115 mm, p=0.001) were more common in patients than controls. Reclassification into the high-risk category was greater in patients (34% vs. 25%, p=0.037). Age (beta coefficient 2.74 [95%CI 1.34-5.62] vs. beta coef. 0.63 (95%CI 0.40-0.99) in patients, interaction p=0.001) and serum LDL-cholesterol (beta coef. 1.03 [95%CI 1.02-1.04] vs. beta coef. 1.00 [0.99-1.01], interaction p=0.029) showed a higher effect on reclassification in controls after multivariable analysis. Although reclassification in axSpA was associated with higher ASDAS-CRP, BASFI and BASMI scores, these associations were lost after adjusting for cardiovascular risk factors. CONCLUSIONS Patients with axSpA are more likely to be reclassified into the very-high risk category after carotid ultrasound than controls. The influence of traditional cardiovascular risk factors on this reclassification differs between patients and controls.OBJECTIVES To systematically evaluate, through a Medline search, the role of omalizumab in eosinophilic granulomatosis with polyangiitis (EGPA). METHODS A systematic review was performed with the following inclusion criteria original articles and case reports written in English and reporting an association between omalizumab and EGPA. RESULTS We found 18 papers on EGPA (14 case reports, 3 retrospective cohort studies, 1 prospective cohort study). Omalizumab showed to be effective as corticosteroid-sparing agent in EGPA patients with severe asthmatic manifestations. On the contrary, conflicting results concerned its use in refractory forms of EGPA. Plausible is the increased risk of EGPA onset among asthmatic patients treated with omalizumab, probably related to steroid reduction, even if it cannot be excluded that omalizumab might be occasionally directly involved in the pathogenesis. CONCLUSIONS Our findings support the use of omalizumab in selected forms of EGPA, but caution in the tapering of corticosteroids is also recommended. Quality of evidence is limited, as the source of information was mainly case reports. Clinical trials are required in order to evaluate the role of omalizumab in EGPA and to ascertain the risk of asthmatic patients given omalizumab to develop EGPA.