The objective of this study was to investigate whether clinical and laboratory data, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis could predict relapse in 30 patients with polyarteritis nodosa (PAN) having the follow-up duration for over 12 months. We reviewed the medical charts of 30 patients with PAN. We obtained clinical and laboratory data at diagnosis, and we compared them between the two groups based on relapse. The optimal cut-off values of BVAS and FFS (1996) at diagnosis to predict relapse were extrapolated. The mean age of patients (15 men) was 50.8 years, and the mean follow-up duration was 64.1 months. Nine patients (30.0%) had experience relapse after remission. Patients having relapse showed the higher frequency of weight loss and ocular symptoms and the less frequency of diastolic hypertension than those having not (p < 0.005 for all). On multivariate logistic regression analysis, weight loss was the only independent predictor of relapse, but on Cox Hazard model analysis, its statistical significance disappeared. The mean initial BVAS and FFS (1996) of patients in relapse group were higher than those of patients in no relapse group (p < 0.005 for all). Patients having initial BVAS over 13.5 and FFS (1996) over 1 exhibited significantly higher risk of relapse than those having not (RR 40.0 and RR 7.0, respectively). However, initial BVAS over 13.5 only remained significant in Kaplan-Meier survival analysis. In conclusion, BVAS over 13.5 at diagnosis was the only independent predictor of relapse of PAN.
We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). The mean age and period from diagnosis to echocardiography were 54.5 years (30 men) and 1.4 months. The mean age of controls was 51.6 years. AAV patients exhibited lower mean left ventricle ejection fraction (LVEF) (64.0 vs. 69.1%, P = 0.002) and higher mean E/E' ratio (11.7 vs. 8.8, P = 0.001) and right ventricle systolic pressure (RVSP) (30.2 vs. 23.2 mm Hg, P < 0.001) than the controls. Each variant of AAV presented a different pattern of echocardiographic features in comparison with controls. MPA patients exhibited systolic and diastolic dysfunctions and pulmonary arterial hypertension more often than controls. Meanwhile, GPA patients exhibited only diastolic dysfunction, and EGPA patients had systolic dysfunction and pulmonary arterial hypertension more frequently than controls. No meaningful differences in echocardiographic features appeared among AAV variants. AAV patients exhibited reduced systolic function and advanced diastolic dysfunctions and pulmonary arterial hypertension near the time of diagnosis compared with controls. Each variant of AAV presented a different pattern in echocardiographic features.
BackgroundLupus pathogenesis is closely associated with interferon gamma (IFN-γ), which plays a central role in innate and adaptive immunity. The aim of this study was to evaluate the ex vivo production of IFN-γ after stimulation of peripheral blood mononuclear cells with phytohemagglutinin (PHA) in patients with lupus, according to disease activity.MethodsThis study included 118 patients with lupus who had undergone IFN-γ-releasing assays (IGRAs) to screen for tuberculosis. Data on IFN-γ production in negative (nil) and positive (mitogen with PHA) controls were collected and analysed. The difference (mitogen minus nil) was used to calculate ex vivo IFN-γ production. Disease activity was evaluated using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K). Poor hospitalisation outcome was defined as in-hospital mortality or intensive care unit admission. Associations among disease activity, poor hospitalisation outcome, and ex vivo IFN-γ production were assessed.ResultsThe level of ex vivo IFN-γ production was significantly lower in patients with active systemic lupus erythematosus (SLE) (n = 64) than in those with inactive SLE (n = 54) (median 0.92 vs. 11.06 IU/mL, p < 0.001). Ex vivo IFN-γ production was correlated with the SLEDAI-2 K (r = − 0.587, p < 0.001). Results of multivariate logistic regression analysis showed that ex vivo IFN-γ production ≤ 7.19 IU/mL was an independent predictor for discriminating active and inactive lupus. In addition, patients with ex vivo IFN-γ production ≤ 0.40 IU/mL had more frequent poor hospitalisation outcomes than those with ex vivo IFN-γ production > 0.40 (40.0% vs. 9.3%, p = 0.001). The proportion of indeterminate IGRA results was higher in patients with active lupus than in those with inactive lupus (45.3% vs. 0.0%, p < 0.001) because of decreased ex vivo IFN-γ production.ConclusionsEx vivo IFN-γ production is a useful biomarker for assessing disease activity and predicting poor clinical outcomes of SLE.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-017-1404-z) contains supplementary material, which is available to authorized users.
Objectives: To investigate the impact of European Scleroderma Trials and Research(EUSTAR) standardized training on the accuracy of modified Rodnan skin score (mRSS) in patients with systemic sclerosis (SSc). Methods:Eight SSc patients (four diffuse, four limited) and 10 physicians (4 fellows, 6 professors) were included. Gold-standard mRSS was performed by a senior instructor. Training comprised a video presentation and a live demonstration. Each physician performed mRSS with no clinical information in all patients: (a) before training; (b) after video session; and (c) after live demonstration. Primary outcome was the change in scoring accuracy, which was defined as the difference from the gold-standard skin score, as analyzed using a linear mixed model. Results: Mean (standard deviation) difference from the gold-standard score in all measurements by participants before the training was 7.7 (9.5). Completion of training significantly enhanced mRSS accuracy (adjusted β = −7.61; 95% CI: −11.91 to −3.32). This was largely attributable to the video presentation (adjusted β = −5.47; −9.16 to −1.78), although the live demonstration was associated with numerical | 97 PARK et Al.
Eighty of 113 patients (81.6%) previously classified by Chuang and Hunder criteria or Healey criteria fulfilled 2012 EULAR/ACR criteria for PMR.
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