The OMERACT core set of outcome measures for use in clinical trials of ANCA-associated vasculitis

There has been a marked increase in the past 15 years in the number and quality of clinical trials in the idiopathic inflammatory vasculitides, especially the small-vessel vasculitides known as antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis [AAV; granulomatosis, with polyangiitis (Wegener's)]. These trials have been conducted by multicenter, international groups in Europe and the United States with financial support provided by government agencies and biopharmaceutical companies. This increased clinical trial activity in vasculitis has been accompanied by the development and validation of new outcome measures--a challenging process for these complex, multiorgan system diseases. The international OMERACT Vasculitis Working Group has developed and implemented an iterative research agenda that has utilized accumulated experience and datasets from several multicenter clinical trials and large cohort studies. This work has led to the development, evaluation, validation, and endorsement, through the OMERACT consensus and validation processes, of a "core set" of outcome measurements for use in clinical trials of AAV. The core set includes domains of disease activity, damage assessment, patient-reported outcomes, and mortality; there is at least one validated outcome measurement instrument available for each domain. This report reviews the domains of illness in AAV included in the OMERACT core set, describes the instruments validated to measure these domains, and presents the approved core set.     

Femoral Vein Wall Thickness Measurement May Be a Distinctive Diagnostic Tool to Differentiate Behçet’s Disease with Intestinal Involvement and Crohn’s Disease

Digestive Diseases and Sciences - Behçet’s disease (BD) and Crohn’s disease (CD) cannot be easily differentiated in young adults presenting with nonspecific gastrointestinal (GI)...     

HADS-depression score is a mediator for illness perception and daily life impairment in Takayasu’s arteritis

Clinical Rheumatology - The aim of this study was to evaluate the relationships among the disease activity, illness perception, daily life performance, anxiety and depression status as potential...     

HSP 60 expression in mucocutaneous lesions of Behçet''s disease

BACKGROUND: Heat shock protein (60 kd HSP) has been implicated in the etiology of Beh?et's disease, but its expression at sites of inflammation is unknown. OBJECTIVE: Our aim was to investigate local HSP 60 expression and to quantify T-cell receptor (TCR) gamma delta-positive cells, which are known to respond to HSP peptides. METHODS: Patients with active Beh?et's disease (n = 21) and controls (n = 18) were included. Flow cytometric analysis was performed on peripheral blood to investigate TCR gamma delta-positive cell counts. Biopsies were performed on active skin lesions, and immunohistochemical analysis was performed by a streptavidin-biotin method using the monoclonal ML-30 antibody; HSP staining intensity and distribution were evaluated in a blinded fashion. Immunohistochemical studies were performed to quantify TCR gamma delta-positive cells at lesional sites. RESULTS: Mucocutaneous lesions of patients with Beh?et's disease had statistically significantly increased expression of HSP 60/65. Peripheral blood TCR gamma delta-positive cell counts were similar in both groups. However, lesional skin of patients with Beh?et's disease had significantly increased gamma delta-positive T-cell counts. CONCLUSION: Up-regulation of HSP expression was found at lesional skin sites in Beh?et's disease. The increased number of TCR gamma delta-positive cells, which are known to respond to HSP peptides, may support the function of HSPs in the etiology of Beh?et's disease. However, these findings may also be an epiphenomenon that needs to be further investigated.     

Mannose binding lectin levels are not related to radiographic damage in ankylosing spondylitis

In the current study we aimed to investigate the effect of MBL deficiency in radiographic damage of the spine in a large group of AS patients. One hundred and ninety-one AS patients and 85 healthy controls were studied. Disease activity, radiological scores, and demographic features were recorded. MBL levels were measured with standard ELISA kits. Results showed that median MBL levels in AS and healthy controls were 2,530 (range 0–5,861) ng/ml and 3,415 (0–7,950) ng/ml, respectively (p = 0.1). MBL deficiency (<500 ng/ml) was comparable in both groups (%21.5 in AS, % 17.6; p = 0.5). Disease activity, clinical picture, and therapies were not associated with MBL levels. Both BASRI and mSASSS scores were found similar in AS patients with or without MBL deficiency [BASRI: MBL < 500 ng/ml: 6(2–12), MBL ≥ 500 ng/ml: 6(2–12); p = 0.75], [mSASSS: MBL < 500 ng/ml: 3(0–72), MBL ≥ 500 ng/ml: 5(0–72); p = 0.81]. We conclude that MBL deficiency prevalence is not increased in AS patients and it is not a cause of a severe radiographic damage.     

The prevalence of metabolic syndrome is increased in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis

Purpose

Cardiovascular disease is one of the major causes of mortality in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Metabolic syndrome (MetS) is associated with increased cardiovascular risk in the normal population. However, MetS in AAV has not been adequately investigated. We aimed to determine MetS prevalence and associated factors in AAV patients.

Methods

Thirty-seven AAV patients and 42 healthy controls were enrolled. MetS was determined by International Diabetes Federation (IDF) and National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATPIII) criteria. The relationship between clinical features of AAV and MetS was also investigated.

Results

MetS was significantly higher in AAV patients than controls by NCEP-ATPIII (51.4% vs. 26.2%, p 0.022) and IDF (62.2% vs. 35.7%, p 0.020). When AAV patients with MetS were compared to those without, there were significant differences in age, CRP, GFR and NT-pro-BNP. Age [58 (13) vs. 50 (8) years p: 0.028], CRP [4.0 (3.6) vs. 3.2 (1.0) mg/l, p 0.021] and NT-pro-BNP [173.5 (343.7) vs. 106.0 (103.0) pg/ml, p 0.013] were significantly higher in AAV patients with MetS than those without; GFR was significantly lower [38 (46) vs. 83 (51) ml/min/1.73 m², p 0.004]. ROC curve analysis showed NT-pro-BNP?>?58.0 ng/ml predicted MetS with 87.1% sensitivity and 46.7% specificity (Area under curve: 0.71, CI 0.536–0.902, p 0.041). Multivariate analysis revealed age [OR (95% CI): 1.180 (1.010–1.370), p 0.039] and NT-pro-BNP?>?58 pg/ml [OR (95% CI): 5.5 (1.02–30.1) p 0.047] were independent predictors of MetS in AAV patients.

Conclusion

MetS is significantly higher in AAV patients than controls and is associated with age and NT-pro-BNP. Screening and treating MetS may improve prognosis in AAV patients.

     

Water self-diffusion in the calf lens

Proton pulsed gradient spin echo (PGSE) nuclear magnetic resonance (NMR) spectroscopy was used to explore the free and restricted non-Brownian nature of lens water self-diffusion in calf lens tissue. At all temperatures investigated the water self-diffusion coefficient (Dw) of the cortical homogenate (25% protein) was 1.6-1.7 times greater than that for the nucleus (42% protein), and 0.3-0.5 times the value of Dw for pure water. The nuclear lens homogenate displayed anomalous temperature dependent water diffusion behavior, i.e. a departure from the smooth monotonic decrease in Dw with decreasing temperature, in the temperature range of 3-5 degrees C. By contrast, no such behavior was observed for cortical homogenate. Analysis of water proton echo attenuation data employing a parallel-plate model of restricted diffusion provided values for the parallel-plate barrier separation and self-diffusion coefficient in the limit of free diffusion. Nuclear material showed a smaller spatially average barrier separation and a significantly stronger barrier separation temperature dependence than cortical material.