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Cornelia M Weyand - One of the best experts on this subject based on the ideXlab platform.
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major histocompatibility complex class i recognizing receptors are disease risk genes in Rheumatoid arthritis
Journal of Experimental Medicine, 2001Co-Authors: Jenghsien Yen, Cornelia M Weyand, Brenda E Moore, Takako Nakajima, Dirk Scholl, Daniel J Schaid, Jorg J GoronzyAbstract:Rheumatoid arthritis (RA) is a heterogeneous syndrome of which a subset of patients develops vascular inflammation. The genetic determinants that confer risk for Rheumatoid Vasculitis are not known, but patients with vascular complications are known to have an expansion of CD4+CD28null T cells, a cell population potentially involved in endothelial damage. CD4+CD28null T cell clones isolated from RA patients with Vasculitis were found to express killer cell immunoglobulin–like receptors (KIRs) with the stimulatory KIR2DS2 often present in the absence of opposing inhibitory receptors with related specificities. To test the hypothesis that the KIR2DS2 gene is involved in the development of Vasculitis, association studies were performed. The KIR2DS2 gene was significantly enriched among patients with Rheumatoid Vasculitis compared with normal individuals (odds ratio 5.56, P = 0.001) and patients with RA but no Vasculitis (odds ratio 7.96, P = 0.001). Also, the distribution of human histocompatibility leukocyte antigen (HLA)-C, the putative ligand for KIRs, was significantly different in patients with Rheumatoid Vasculitis in comparison with the control populations. These data suggest that HLA class I–recognizing receptors and HLA class I genes are genetic risk determinants that modulate the pattern of RA expression. Specifically, KIR2DS2 in conjunction with the appropriate HLA-C ligand may have a role in vascular damage by regulating CD4+CD28null T cells.
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Rheumatoid Vasculitis manifesting as intra abdominal hemorrhage
Mayo Clinic Proceedings, 1995Co-Authors: Antonio A Achkar, Anthony W Stanson, Michael C Johnson, Sanjay S Srivatsa, Lowell C Dale, Cornelia M WeyandAbstract:Rheumatoid Vasculitis, an extra-articular component of Rheumatoid arthritis, causes a wide spectrum of manifestations that range from clinically insignificant to life-threatening disease. As a systemic necrotizing arteritis, Rheumatoid Vasculitis is usually characterized by end-organ ischemia. Herein we describe a patient with abdominal pain and syncope due to intraabdominal hemorrhage from a ruptured aneurysm of the inferior pancreaticoduodenal artery in the setting of Rheumatoid Vasculitis. Although the intra-abdominal hemorrhage was the unusual manifestation of Rheumatoid Vasculitis in this patient, he had a history of prior extra-articular Rheumatoid disease, including pulmonary fibrosis and Sjogren's syndrome with associated parotid Iymphoproliferative disease. In patients with Rheumatoid arthritis who have abdominal pain and an unexplained rapidly decreasing hemoglobin concentration, the diagnosis of intra-abdominal hemorrhage from a ruptured visceral aneurysm due to Rheumatoid Vasculitis should be considered, even in the absence of other indications of systemic Vasculitis.
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Vasculitis in Rheumatoid arthritis
Current Opinion in Rheumatology, 1994Co-Authors: Jorg J Goronzy, Cornelia M WeyandAbstract:The inflammatory process characteristic of Rheumatoid arthritis is typically targeted to the synovial membrane. In a subset of patients, Rheumatoid disease is complicated by an inflammatory destruction of blood vessels, Rheumatoid Vasculitis. Rheumatoid Vasculitis has been understood to be the result of severe disease extending beyond the joint, possibly caused by immune complex deposition. In an alternative pathogenetic model, it is hypothesized that Rheumatoid Vasculitis represents a distinct dimension of Rheumatoid disease. This model implies that Rheumatoid arthritis patients can be separated into two groups: individuals with synovial disease and individuals with synovial plus extra-articular disease. The model is supported by immunogenetic analysis describing an accumulation of Vasculitis patients with Rheumatoid arthritis among HLA-DRB1*0401 homozygotes. The clinical experience that different types of extra-articular diseases cluster in some patients and the lack of correlation between the activity of synovial and extra-articular disease provide further support for this model.
Richard A Watts - One of the best experts on this subject based on the ideXlab platform.
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Rheumatoid arthritis Rheumatoid Vasculitis down but not out
Nature Reviews Rheumatology, 2014Co-Authors: Richard A WattsAbstract:Rheumatoid Vasculitis is one of the most severe complications of Rheumatoid arthritis. New data suggests that hydroxychloroquine and low-dose aspirin might be protective, but that there remains notable mortality despite intensive immunosuppression with cyclophosphamide or biologic agents.
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systemic Rheumatoid Vasculitis in the era of modern immunosuppressive therapy
Rheumatology, 2014Co-Authors: Eleana Ntatsaki, J Mooney, David G I Scott, Richard A WattsAbstract:Objectives. Systemic Rheumatoid Vasculitis (SRV) is a rare but potentially serious systemic disease manifestation of Rheumatoid arthritis (RA) characterized by the development of necrotizing Vasculitis. The incidence of SRV appears to be decreasing possibly reflecting progress in RA treatment. The aims of this study were to review the clinical manifestations of SRV in a stable well-defined population during 200110 and to compare with our previous cohort (19882000) and also a cohort from 1975 to 1981. Methods. Using Norfolk Vasculitis Register, a prospective register of patients with systemic Vasculitis since 1988, all patients with a diagnosis of SRV from 1 January 2001 until 31 December 2010 were identified. SRV was defined according to the Scott and Bacon criteria (1984). Clinical features were obtained by retrospective case note review. Results. Eighteen patients with SRV were identified (10 male), median age at diagnosis was 72 years and average disease duration 15.6 years. The average annual incidence for 200110 was 3.9 per million. Oneyear mortality was 12% and 5-year mortality 60%. The clinical manifestations were similar apart from systemic and cutaneous features which were more common in the earlier cohorts. Conclusion. The incidence of SRV has declined significantly in the last 40 years; but the clinical manifestations remain similar. Systemic symptoms, and cutaneous manifestations such as infarcts and nodules, are slightly less common in the recent cohort. Despite modern immunosuppressive therapy the prognosis remains poor.
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Rheumatoid Vasculitis becoming extinct
Rheumatology, 2004Co-Authors: Richard A Watts, J Mooney, S E Lane, Dgi ScottAbstract:Background. Systemic Rheumatoid Vasculitis (SRV) is a relatively rare complication of RA. The incidence of SRV appeared to increase during the 1970s and 1980s from 6.0 to 12.5/million. During the 1990s there have been major changes in the treatment of RA, with more aggressive control of inflammation. Our aim was to study the epidemiology of SRV in a stable, well-defined population over a 15-yr period. Methods. Since 1988 we have maintained a prospective register of all patients with systemic Vasculitis attending the Norfolk and Norwich University Hospital. Patients presenting with new-onset SRV, as defined by the criteria of Scott and Bacon, and registered with general practitioners in the former Norwich Health Authority area between 1988 and 2002 were identified. The population in 2002 was estimated to be 445 000 (215 000 males). Results. Fifty-one patients (24 male) with SRV were identified, with median age 61 yr and disease duration 16.8 yr. The overall annual incidence was 7.9/million (95% CI 5.9–10.4) (males, 7.7/million; females, 8.1/million). During the first quinquennium (1988–92) the incidence was 11.6/million (95% CI 7.4–17.0) and during the third (1998–2002) it was 3.6/million (95% CI 1.6–7.1). A rolling 3-yr average showed that the peak incidence was in 1992–94, at 15.2/million (95% CI 9.1–23.8), and the nadir was in 1998–2000, at 3.0/million (95% CI 0.8–7.8). A similar pattern was seen for males and females. There was no difference in age or disease duration at onset of SRV between the three quinquennia. Conclusions. The incidence of SRV has declined dramatically since the 1980s. This could be due to better control of inflammatory disease or changes in smoking habits.
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the incidence of Rheumatoid Vasculitis in the norwich health authority
Rheumatology, 1994Co-Authors: Richard A Watts, David M Carruthers, Deborah P M Symmons, Dgi ScottAbstract:Systemic Rheumatoid Vasculitis (SRV) is an uncommon but potentially serious complication of RA. The incidence of SRV is unknown. The aim of this study was to estimate the incidence of SRV in the UK. Over a 6-yr period vigorous attempts were made to identify all patients with newly diagnosed SRV living within the Norwich Health Authority (NHA). The overall annual incidence of SRN is 12.5/million (95% CI: 8.5-17.7). The annual incidence rate for men was 15.8/million (95% CI: 9.5-24.7) and 9.41/million (95% CI: 4.8-16.4) for women. The incidence of SRN in this study is higher than previous estimates
Tsuyoshi Kasama - One of the best experts on this subject based on the ideXlab platform.
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elevated serum levels of macrophage migration inhibitory factor and their significant correlation with Rheumatoid Vasculitis disease activity
Modern Rheumatology, 2012Co-Authors: Kuninobu Wakabayashi, Takeo Isozaki, Tsuyoshi Odai, Nobuyuki Yajima, K Otsuka, Michihito Sato, Ryo Takahashi, Yusuke Miwa, Tsuyoshi KasamaAbstract:Macrophage migration inhibitory factor (MIF) is recognized to be an important mediator in several inflammatory disorders, including Rheumatoid arthritis (RA) and Vasculitis. To evaluate the role of MIF in Rheumatoid Vasculitis (RV), we determined serum levels of MIF by enzyme-linked immunosorbent assay in RA patients with and without Vasculitis and assessed their relationship to disease activity. Serum was obtained from 95 RA patients during active disease states [49 without Vasculitis, 35 with extra-articular manifestations without histologically proven Vasculitis, and 11 with histologically proven Vasculitis] and from 22 healthy individuals. Vasculitis disease activity was assessed using the Birmingham Vasculitis Activity Score (BVAS). MIF levels were significantly higher in RA patients than in controls. Moreover, MIF levels were significantly higher in RA patients with Vasculitis than in those without vasculitic complications. In all RA patients, a statistically significant positive correlation was observed between serum MIF levels and each of the following: serum levels of C-reactive protein, Rheumatoid factor, and thrombomodulin; and the erythrocyte sedimentation rate. In the RV group, the elevation of MIF levels correlated with the BVAS. Our findings suggest that MIF may serve as an additional serologic inflammatory marker of disease activity in RV, and it may be implicated in the pathogenesis of RV.
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increased serum levels of soluble fractalkine cx3cl1 correlate with disease activity in Rheumatoid Vasculitis
Arthritis & Rheumatism, 2006Co-Authors: Mizuho Matsunawa, Takeo Isozaki, Tsuyoshi Odai, Nobuyuki Yajima, Hiroko Takeuchi, Masao Negishi, Hirotsugu Ide, Mitsuru Adachi, Tsuyoshi KasamaAbstract:Objective To determine levels of soluble fractalkine (sFkn) in Rheumatoid arthritis (RA) patients with and without Rheumatoid Vasculitis (RV), and to assess the relationship of sFkn levels to disease activity. Methods Serum was obtained from 98 RA patients (54 without Vasculitis, 36 with extraarticular manifestations but without histologically proven Vasculitis, and 8 with histologically proven Vasculitis) and from 38 healthy individuals. Levels of sFkn were measured by enzyme-linked immunosorbent assay. Expression of Fkn and CX3CR1 was quantified by real-time polymerase chain reaction. Vasculitis disease activity was assessed using the Birmingham Vasculitis Activity Score and the Vasculitis Activity Index. Results Serum sFkn levels were significantly higher in patients with RA than in controls and were significantly higher in RA patients with RV than in those without vasculitic complications. Statistically significant correlations were observed between serum sFkn levels in RA patients and levels of C-reactive protein, Rheumatoid factor, immune complex, and complement. In the RV group, sFkn levels also correlated with disease activity. Immunohistochemical analysis indicated that Fkn levels were associated mainly with endothelial cells in vasculitic arteries. In addition, expression of CX3CR1 messenger RNA was significantly greater in peripheral blood mononuclear cells from patients with active RV than in those from other RA patients or controls. Notably, serum sFkn levels were significantly diminished following successful treatment and clinical improvement. Conclusion These findings suggest that Fkn and CX3CR1 play crucial roles in the pathogenesis of RV and that sFkn may serve as a serologic inflammatory marker of disease activity in RA patients with Vasculitis.
Alexandre E Voskuyl - One of the best experts on this subject based on the ideXlab platform.
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diagnostic strategy for the assessment of Rheumatoid Vasculitis
Annals of the Rheumatic Diseases, 2003Co-Authors: Alexandre E Voskuyl, Aeilko H Zwinderman, Johanna M W Hazes, E M Paleolog, F J M Van Der Meer, M R Daha, Ferdinand C BreedveldAbstract:Objective: To determine the clinical features associated with histologically proven Rheumatoid Vasculitis (HRV) and the additional diagnostic value of serological markers in an inception cohort of 81 patients with Rheumatoid arthritis (RA) suspected of RV. Methods: The presence and number of recently developed extra-articular manifestations (EAMs) and a weighted EAM score, as well as the levels of serological markers, were compared between 31 patients with RA with histologically proven Vasculitis and 50 patients with RA in whom Vasculitis could not be documented histologically. The following markers were evaluated: circulating immune complexes, complement components C3 and C4, class-specific Rheumatoid factors (IgM RF, IgG RF, IgA RF), antineutrophil cytoplasmic antibodies, antinuclear antibodies, antiendothelial antibodies, circulating intercellular adhesion molecule-1 and -3, circulating vascular cell adhesion molecule and E-selectin, cellular fibronectin, von Willebrand factor antigen, and C reactive protein. The diagnostic value of these markers, in addition to the clinical features, was evaluated with logistic regression analysis. Results: Peripheral neuropathy or purpura/petechiae, or both, were the most important clinical features to discriminate patients with RA with and without histologically proven RV. The presence of a high number of EAMs and a higher weighted EAM score in patients with RA suspected of Vasculitis were also associated with an increased probability of histologically proven RV. After adjustment for EAMs, only the combination of an increased serum IgA RF level and a decreased serum C3 level appeared to make an additional contribution to the diagnosis histologically proven RV. Evidence of systemic Vasculitis was found in a muscle biopsy of the rectus femoris in 9/14 (64%) patients with Vasculitis with neuropathy and in 3/11 (27%) patients with purpura/petechiae and Vasculitis of the skin. Conclusions: In the diagnostic process of RV the presence of peripheral neuropathy and/or purpura/petechiae or a high weighted EAM score will increase the probability of histologically proven RV. Of the circulating factors previously suggested to be markers for RV only IgA RF and C3 further increase the probability of histologically proven RV and may be useful to guide diagnostic decisions.
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the diagnostic value of perivascular infiltrates in muscle biopsy specimens for the assessment of Rheumatoid Vasculitis
Annals of the Rheumatic Diseases, 1998Co-Authors: Alexandre E Voskuyl, S G Van Duinen, A H Zwinderman, F C Breedveld, J M W HazesAbstract:OBJECTIVE To determine the diagnostic value of perivascular infiltrates (PVI) in randomly obtained muscle biopsy specimens for the assessment of Rheumatoid Vasculitis (RV). METHODS The number and size of PVIs, defined as the presence of mononuclear or polymorphonuclear cells around ⩾ 50% of the circumference of a vessel wall, as well as the presence of fibrinoid necrosis were determined in frozen sections of muscle samples of RV patients with histologically confirmed Vasculitis in fixed muscle tissue (n=12). The findings were compared with those observed in frozen sections of muscle biopsy specimens of Rheumatoid arthritis (RA) patients not suspected of Vasculitis (n=14) and patients with osteoarthritis (OA) (n=11). The presence of PVIs and of fibrinoid necrosis were sought in four frozen sections of the muscle biopsy specimen. RESULTS PVIs were observed in 75% of the RV patients, which was significantly (p CONCLUSIONS The assessment of PVIs with ⩾ three cell layers in a muscle biopsy specimen is a specific and reliable test in discriminating RV from RA without Vasculitis. The demonstration in muscle of PVIs with ⩾ three cell layers is more sensitive than that of fibrinoid necrosis in the diagnosis of RV.
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factors associated with the development of Vasculitis in Rheumatoid arthritis results of a case control study
Annals of the Rheumatic Diseases, 1996Co-Authors: Alexandre E Voskuyl, Marie Louise Westedt, J P Vandenbroucke, A H Zwinderman, F C Breedveld, J M W HazesAbstract:OBJECTIVE: To investigate those characteristics of patients with Rheumatoid arthritis (RA) that are associated with the development of Rheumatoid Vasculitis (RV). METHODS: Demographic and clinical data of 69 patients who had been diagnosed as having RV were compared with those of 138 contemporaneous control patients with RA who were not suspected to have Vasculitis. Vasculitis was confirmed histologically in 96% of the subjects with RV. RESULTS: Variables associated with the development of RV were: 1) male gender, presence of increased serum concentrations of Rheumatoid factor, joint erosions, subcutaneous nodules, number of disease modifying antirheumatic drugs previously prescribed, treatment (ever) with D-penicillamine or azathioprine; 2) presence of nail fold lesions and any other extrarticular feature one year before the time of diagnosis of RV; 3) treatment with corticosteroids at the time of diagnosis of RV. CONCLUSIONS: The development of RV is associated with male gender, extra-articular features, and a severe course of RA as indicated by the presence of joint destruction and need for intensive treatment with antirheumatic drugs. The strongest association was found with the presence of increased concentrations of Rheumatoid factor.
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the mortality of Rheumatoid Vasculitis compared with Rheumatoid arthritis
Arthritis & Rheumatism, 1996Co-Authors: Alexandre E Voskuyl, Ferdinand C Breedveld, Aeilko H Zwinderman, Marie Louise Westedt, J P Vandenbroucke, Johanna M W HazesAbstract:Objective. To determine whether the mortality of patients with Rheumatoid Vasculitis (RV) is increased in comparison with that of patients with Rheumatoid arthritis (RA). Methods. The mortality of all RV patients identified in 1980–1992 (n = 61) was compared with that of 244 RA controls matched for the year the diagnosis was made in the RV cases. Hazard ratios (HR) of death were calculated with a multivariate survival analysis, adjusting for age, sex, comorbidity, treatment, and parameters of RA severity. Results. The unadjusted risk of death (HR) in RV patients compared with RA controls was 1.65 (95% confidence interval [95% CI] 1.05–2.58). After adjustment for prognostic factors, the HR was reduced to 1.26 (95% CI 0.79–2.01), mainly due to removal of the effects of age and sex. No excess mortality was seen in RV patients with severe organ involvement when compared with RV patients without severe organ involvement, although the former patients were treated more often with cytostatic and immunosuppressive drugs. Infection was the main cause of death in the RV patients, and cardiovascular disease in the RA controls. Vasculitis was reported as the cause of death in only 1 RV patient. Conclusion. After allowance for general risk factors such as age and sex, there remains only a slight excess mortality in RV patients compared with RA controls.
J M W Hazes - One of the best experts on this subject based on the ideXlab platform.
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the diagnostic value of perivascular infiltrates in muscle biopsy specimens for the assessment of Rheumatoid Vasculitis
Annals of the Rheumatic Diseases, 1998Co-Authors: Alexandre E Voskuyl, S G Van Duinen, A H Zwinderman, F C Breedveld, J M W HazesAbstract:OBJECTIVE To determine the diagnostic value of perivascular infiltrates (PVI) in randomly obtained muscle biopsy specimens for the assessment of Rheumatoid Vasculitis (RV). METHODS The number and size of PVIs, defined as the presence of mononuclear or polymorphonuclear cells around ⩾ 50% of the circumference of a vessel wall, as well as the presence of fibrinoid necrosis were determined in frozen sections of muscle samples of RV patients with histologically confirmed Vasculitis in fixed muscle tissue (n=12). The findings were compared with those observed in frozen sections of muscle biopsy specimens of Rheumatoid arthritis (RA) patients not suspected of Vasculitis (n=14) and patients with osteoarthritis (OA) (n=11). The presence of PVIs and of fibrinoid necrosis were sought in four frozen sections of the muscle biopsy specimen. RESULTS PVIs were observed in 75% of the RV patients, which was significantly (p CONCLUSIONS The assessment of PVIs with ⩾ three cell layers in a muscle biopsy specimen is a specific and reliable test in discriminating RV from RA without Vasculitis. The demonstration in muscle of PVIs with ⩾ three cell layers is more sensitive than that of fibrinoid necrosis in the diagnosis of RV.
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factors associated with the development of Vasculitis in Rheumatoid arthritis results of a case control study
Annals of the Rheumatic Diseases, 1996Co-Authors: Alexandre E Voskuyl, Marie Louise Westedt, J P Vandenbroucke, A H Zwinderman, F C Breedveld, J M W HazesAbstract:OBJECTIVE: To investigate those characteristics of patients with Rheumatoid arthritis (RA) that are associated with the development of Rheumatoid Vasculitis (RV). METHODS: Demographic and clinical data of 69 patients who had been diagnosed as having RV were compared with those of 138 contemporaneous control patients with RA who were not suspected to have Vasculitis. Vasculitis was confirmed histologically in 96% of the subjects with RV. RESULTS: Variables associated with the development of RV were: 1) male gender, presence of increased serum concentrations of Rheumatoid factor, joint erosions, subcutaneous nodules, number of disease modifying antirheumatic drugs previously prescribed, treatment (ever) with D-penicillamine or azathioprine; 2) presence of nail fold lesions and any other extrarticular feature one year before the time of diagnosis of RV; 3) treatment with corticosteroids at the time of diagnosis of RV. CONCLUSIONS: The development of RV is associated with male gender, extra-articular features, and a severe course of RA as indicated by the presence of joint destruction and need for intensive treatment with antirheumatic drugs. The strongest association was found with the presence of increased concentrations of Rheumatoid factor.