Guldagerhurst2246

Subgroup analysis showed that CNI were effective for AOSD patients with high ALT level (hazard ratio 0.11, 95% confidential interval 0.02-0.59) and severe complications (hazard ratio 0.11, 95% confidential interval 0.01-0.94). The persistency rate of CNI was 71% at 5th year. Adverse events occurred more frequently in the CNI group (18% versus 8%, p=0.02); however, CNI did not involve in increased risk of adverse events, including nephrotoxicity, after adjustment (p=0.23). CONCLUSIONS Our retrospective analysis suggested that CNI could be an effective and safe option for treating AOSD.OBJECTIVES To evaluate the performance of the 2019 European League against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) in Asian patients. METHODS We conducted an electronic medical chart review of patients with SLE and defined rheumatic diseases. Classification criteria of the 1997 ACR, 2012 Systemic Lupus International Collaborating Clinics (SLICC), and 2019 EULAR/ACR were examined based on sensitivity, specificity, positive predictive value, negative predicted value, and accuracy using clinical diagnosis as the gold standard. RESULTS A total of 335 SLE patients and 337 non-SLE patients were analysed. Non-SLE patients included rheumatoid arthritis (RA) (n=92), anti-phospholipid syndrome (APS) (n=57), mixed connective tissue disease (n=52), systemic sclerosis (n=43), primary Sjögren's syndrome (SS) (n=39), undifferentiated connective tissue disease (n=28), RA with secondary SS (n=24), dermatomyositis (n=1), and spondyloarthropathy (n=1). The sensitivity was 97.6% (95% confidence interval (CI) 0.954-0.989) for the 2019 EULAR/ACR criteria, 98.5% (95% CI 0.966-0.995) for the 2012 SLICC criteria and 95.5% (95% CI 0.927-0.975) for the 1997 ACR criteria. The specificity was 91.4% (95% CI 0.879-0.942) for the 2019 EULAR/ACR criteria, 92.6% (95% CI 0.892-0.951) for the 2012 SLICC criteria 93.8% (95% CI 0.906-0.961) for the 1997 ACR criteria. CONCLUSIONS The 2019 EULAR/ACR criteria for SLE had comparable performance to the 2012 SLICC criteria regarding diagnostic sensitivity and specificity in Korean population of SLE and other rheumatic diseases. However, the new criteria could not reach higher specificity than the 2012 SLICC criteria.OBJECTIVES To identify the predictors of coronary involvement, and to determine the impact of coronary involvement on long-term outcomes in patients with Takayasu's arteritis (TAK). METHODS This retrospective cohort study of TAK patients with coronary evaluation by angiography or computed tomography angiography was conducted in a tertiary center between 1990 and 2018. Risk factors for coronary involvement and predictors of overall survival, cardiovascular event-free survival, and relapse-free survival were investigated. RESULTS The median follow-up was 4.3 years (IQR 2.8-7.1). Out of 130 consecutive TAK patients, 71 (54.6%) had coronary involvement. Multivariate analysis revealed that age (OR 1.537 per 10-year increase, 95% CI 1.176-2.009, p=0.002) and type V angiographic classification (OR 3.449, 95% CI 1.600-7.437, p=0.002) were independent predictors of coronary involvement. Coronary involvement (HR 8.358, 95% CI 1.887-37.033, p=0.015), left ventricular systolic dysfunction (HR 3.889, 95% CI 1.467-10.311, p=0.006), and aortic regurgitation (HR 3.373, 95% CI 1.209-9.408, p=0.020) were independent predictors of overall survival. Furthermore, coronary involvement and baseline active disease were independently associated with increased major cardiovascular events (HR 10.333, 95% CI 2.326-45.906, p=0.017; HR 7.084, 95% CI 1.677-29.914, p=0.008, respectively) and relapse (HR 5.186, 95% CI 2.381-11.295, p less then 0.001; HR 5.694, 95% CI 2.022-16.031, p=0.001, respectively). No immunosuppressive therapy was independently associated with increased cardiovascular events (HR 2.560, 95% CI 1.181-5.550, p=0.002). CONCLUSIONS Coronary involvement is an important predictor of poor long-term outcomes in patients with TAK. Increasing age and type V angiographic classification can help to identify TAK patients with coronary involvement.OBJECTIVES To determine the potential predictive value in patients with systemic lupus erythematous of the ankle-brachial index (ABI) for the occurrence of arterial vascular events. METHODS 216 lupus patients from a prospective clinical cohort were evaluated using the ABI at the start of the study and then followed up for 5 years. Abnormal ABI was defined as an index ≤0.9 or >1.4. Several potential vascular risk factors were also evaluated. Arterial vascular events (AVE) coronary events, cerebrovascular events, peripheral arterial disease and death related to vascular disease. Survival analysis was performed using a competitive risk regression approach, considering non-vascular death as a competitive event. RESULTS 18 arterial events and 14 deaths were identified. In the competitive risk regression analysis, independent predictors of higher risk were identified family history of early AVE [subdistribution hazard ratio (SHR) 5.44, 95% confidence interval (CI) 1.69-17.50, p=0.004)], cumulative prednisone (grams) (SHR 1.01, 95% CI 1.01-1.03, p=0.007) and a personal history of arterial thrombosis (SHR 5.44, 95% CI 1.45-14.59, p=0.004). Female gender was a protective factor (SHR 0.22, 95% CI 0.07-0.77, p=0.017). A statistical trend was detected with abnormal ABI (SHR 2.65, 95% CI 0.86-8.14, p=0.089). CONCLUSIONS Male gender, exposure to high cumulative doses of prednisone, family history of early arterial vascular disease and occurrence of previous arterial thrombosis are independent risk predictors of arterial vascular events in patients with systemic lupus erythematosus. Abnormal ABI may be related to high risk for arterial vascular events.OBJECTIVES This study aims to characterise the clinical phenotype and autoantibody associations in an autoimmune population positive for anti-Ro52 and/or anti-Ro60 autoantibodies. METHODS 508 sera of individuals tested for autoantibody presence were found positive for anti-Ro52 and/or anti-Ro60. Medical records were available for 272 of them. Correlations of clinical, laboratory and other autoantibodies as well as disease phenotypes with the presence of anti-Ro52 and/or anti-Ro60 reactivity were examined. RESULTS Combined serum anti-Ro52/anti-Ro60 reactivity was the most frequent one, mostly seen in Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE) patients. Curaxin137 In thesepatients this reactivity strongly associated with anti-La and/or anti-dsDNA autoantibodies. SS patients with combined anti-Ro52/anti-Ro60 and anti-La reactivity had clinical and/or laboratory risk factors for lymphoma development. link2 Solo anti-Ro52 reactivity was primarily found in idiopathic inflammatory myopathies (IIM), primary biliary cholangitis (PBC), rheumatoid arthritis (RA) and SS patients. Solo anti-Ro52 also associated with anti-Jo1 and anti-M2 autoantibodies and with interstitial lung disease (ILD) in a context of IIM-related lung injury. ILD patients with combined anti-Ro52/anti-Ro-60 reactivity were diagnosed mostly as RA and/or SS. Solo anti-Ro60 reactivity strongly correlated with oral ulcers and co-existed with autoantibodies to Sm and nRNP/Sm. CONCLUSIONS Testing for autoantibodies against both Ro peptides may aim diagnosis, classify clinical manifestations in disease entities and define prognosis in certain autoimmune disorders. A distinct weight could be given to the isolated anti-Ro specificities in the SS classification criteria.OBJECTIVES To compare clinical characteristics, treatment and prognosis of two population-based cohorts of patients with biopsy-proven giant cell arteritis (GCA) from Olmsted County, Minnesota, USA (Olmsted cohort) and the Reggio Emilia area, Northern Italy (Reggio cohort). METHODS All patients residing in Olmsted County and the Reggio Emilia area with a new diagnosis of biopsy-proven GCA in 1986-2007 were retrospectively identified. Patients were followed from GCA diagnosis to death, migration or September 2011. RESULTS The study included 110 patients in the Olmsted and 144 in the Reggio cohort. Compared with the Olmsted cohort, patients from the Reggio cohort had longer duration of symptoms prior to diagnosis (median 1.4 months vs. 0.7, p less then 0.001) and were younger (mean 74.6 years vs. 77.8, p=0.002), more likely to have cranial symptoms (93% vs. 86%, p=0.048), permanent vision loss (21% vs. 6%, p=0.001) and systemic symptoms (67% vs. 46%, p=0.001). ESR and CRP were higher (mean 88 mm/h vs. 73, and 89.0 mg/L vs. 35.2, both p less then 0.001) in the Reggio cohort. Patients from the Olmsted cohort received a higher initial prednisone dose (mean 53.6 mg/day vs. 49.5, p=0.001). There were no differences in relapse rates, cumulative glucocorticoid (GC) dosages at 1, 2 and 5 years, and time to first GC discontinuation. CONCLUSIONS Geographical, genetic and/or environmental factors may contribute to the different clinical features at onset of GCA observed in this study.OBJECTIVES Fibromyalgia (FM) is the most common chronic pain disease in middle-aged women. Patients may also complain of migraine, irritable bowel syndrome and depression, which seriously affect their work and life, causing huge economic losses to society. However, the pathogenesis of FM is still controversial and the effect of the current treatment is far from satisfactory. METHODS Differentially expressed genes (DEGs) and miRNAs (DEMs) were found between FM and normal blood samples. The pathway and process enrichment analysis of the genes were performed. link3 Protein-protein interaction network were constructed. Hub genes were found and analysed in The Comparative Toxicogenomics Database. RESULTS A total of 102 genes were up-regulated and 46 down-regulated, 206 miRNAs down-regulated, and 15 up-regulated in FM. CD38, GATM, HDC, FOS were found as canditate genes. These genes were significantly associated with musculoskeletal disease, mental disorder, immune system disease. There was partial overlap between metformin therapy-related genes and FM-related genes. CONCLUSIONS We found DEGs and DEMs in FM patients through bioinformatics analysis, which may be involved in the occurrence and development of FM and serve as potential targets for diagnosis and treatment.OBJECTIVES Immunoglobulin (Ig) A vasculitis affects children more commonly than adults and previous literature lacks any formal damage assessment. Our aim in this study is to investigate the disease course, relapse rates and prognostic factors in adult patients with IgA vasculitis and to evaluate the disease-related damage. METHODS We assembled a retrospective cohort of adult IgA vasculitis from six tertiary Rheumatology Centres in Turkey. The demographics, clinical characteristics, treatment and outcomes of patients were abstracted from medical records. RESULTS The study included 130 (male/female 85/45) patients and the mean age was 42.2±17 years. Cutaneous manifestations and arthritis/arthralgia were the most common clinical manifestations. One hundred thirteen patients (86.9%) were treated with oral glucocorticoids (GC). As additional immunosuppressive (IS) agents, azathioprine was given to 44 (34.9%) and pulse cyclophosphamide to 18 (12.6%) patients. Seventy-nine patients (60%) had follow-up of median 15 (IQR 7-40) months.