Research News
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Author: Kevin Deane, MD
Date Published: February-2012
Source: Medscape Today
ulmonary arterial hypertension (PAH) is a leading cause of mortality in patients with systemic sclerosis (SSc).[1] Once commonly thought to be a late complication of SSc, support for PAH as an early manifestation of disease is growing. PAH is present within 5 years of the onset of the first non-Raynaud symptoms of SSc in approximately 50% of patients. |
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Author: C. Beyer, N. Reich, S. Schindler, et al
Date Published: January-2012
Source: Annals of the Rheumatic Diseases
Fibrosis and vascular disease are cardinal features of systemic sclerosis (SSc). Stimulators of soluble guanylate cyclase (sGC) are vasoactive drugs that are currently being evaluated in phase III clinical trials for pulmonary arterial hypertension. |
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Author: Richard-Miceli C, Criswell LA.
Date Published: January-2012
Source: Genome Medicine
Most of the recently identified autoimmunity loci are shared among multiple autoimmune diseases. The pattern of genetic association with autoimmune phenotypes varies, suggesting that certain subgroups of autoimmune diseases are likely to share etiological similarities and underlying mechanisms of disease. |
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Author: Benza RL, Miller DP, Barst RJ, et al
Date Published: January-2012
Source: Chest
The Registry to EValuate Early And Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) was established to characterize the clinical course, treatment, and predictors of outcomes in patients with pulmonary arterial hypertension (PAH) in the US. To date, estimated survival based on time of patient enrollment has been established and reported. |
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Author: Dieudé P, Bouaziz M, Guedj M, et al
Date Published: December-2011
Source: Arthritis & Rheumatism
Several autoimmune disorders, including systemic sclerosis (SSc), are characterized by a strong sex bias. To date, it is not known whether genes on the sex chromosomes influence SSc susceptibility. Recently, an IRAK1 haplotype that contains the 196Phe functional variant (rs1059702), located on Xq28, was found to confer susceptibility to systemic lupus erythematosus (SLE). |
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Author: Haines P, Hant FN, Lafyatis R, Trojanowska M, Bujor AM.
Date Published: January-2012
Source: Journal of Cellular and Molecular Medicine
Previous studies have shown that the TGFβ/Alk1/Smad1 signaling pathway is constitutively activated in a subset of systemic sclerosis (SSc) fibroblasts and this pathway is a critical regulator of CCN2 gene expression. Caveolin-1 (cav-1), an integral membrane protein and the main component of caveolae, has also been implicated in SSc pathogenesis. |
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Author: M. Singh, P. Clements, D. Furst, et al
Date Published: January-2012
Source: Arthritis & Rheumatism
One hundred sixty-two patients completed the Work Productivity Survey. Patients indicated whether or not they were employed outside of the home, how many days per month they missed work (employment or household work) due to SSc, and how many days per month productivity was decreased by ≥50%. |
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Author: NIAMS / Tourkina E, et al
Date Published: January-2012
Source: NIAMS Spotlight on Research 2012
Investigators, partially supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases, have found that a deficiency in the protein caveolin-1 (cav-1) is linked to the development of interstitial lung disease, the scarring of lung tissue that causes disability and death in people with scleroderma. |
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Author: Happonen KE, Saxne T, Geborek P, et al
Date Published: January-2012
Source: Arthritis Research & Therapy
Cartilage oligomeric matrix protein (COMP) is found at elevated concentrations in sera of patients with joint diseases such as rheumatoid arthritis (RA) and osteoarthritis (OA). We recently showed that COMP activates complement via the alternative pathway and that COMP-C3b complexes are present in sera of RA patients, but not healthy controls. |
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Author: Yiu KH, Schouffoer AA, Marsan NA, et al
Date Published: December-2011
Source: Arthritis & Rheumatism
Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular inflammation and fibrosis. Visceral involvement, including cardiac manifestations, can lead to severe clinical complications, such as congestive heart failure, arrhythmias, and sudden death. |
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Author: Barnes, J.; Mayes, M.
Date Published: January-2012
Source: Current Opinion in Rheumatology
SSc disease occurrence data are now available for Argentina, Taiwan, and India and continue to show wide variation across geographic regions. The survival rate is negatively impacted by older age of onset, male sex, scleroderma renal crisis, pulmonary fibrosis, pulmonary arterial hypertension, cancer, and antitopoisomerase and anti-U1 antibodies. |
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Author: Imura Y, Shirai Y, Nojima T, et al
Date Published: January-2012
Source: Modern Rheumatology
OBJECTIVES: The anti-Wa antibody found in systemic sclerosis patients reacts with a transfer RNA (tRNA)-associated 48-kDa protein and immunoprecipitates several tRNAs. We investigated the Wa antigen and its binding to tRNA species. |
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Author: K. Sullivan, F. Wigley, C. Denton, J. van Laar, D. Furst
Date Published: January-2012
Source: The Lancet
Richard Burt and colleagues (Aug 6, p 498)1 propose, on the basis of a single-centre trial of 19 patients, that autologous haemopoietic stem-cell transplantation (HSCT) might be the new standard of care for diffuse systemic sclerosis. Although the study strongly supports the use of immunosuppression in systemic sclerosis, several limitations are to be noted. |
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Author: Maurer B, Distler O.
Date Published: January-2012
Source: Clinical Rheumatology
Systemic sclerosis (SSc) is a multisystemic fibrotic disorder that affects the skin and internal organs. Despite an improved outcome probably reflecting a better management of disease complications, morbidity and mortality remain higher than those of patients with other connective tissue diseases. |
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Author: Koschik RW, Fertig N, Lucas MR, et al
Date Published: January-2012
Source: Clinical & Experimental Rheumatology
OBJECTIVES: To compare systemic sclerosis (SSc) patients with and without anti-PM-Scl antibody. METHODS: We reviewed the medical records of 76 anti-PM-Scl antibody positive SSc patients and 2349 anti-PMScl negative SSc patients first evaluated during 1980-2004. |
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Author: M. Rosenblum, I. Gratz, J. Paw, K. Lee, A. Marshak-Rothstein
& A. Abbas
Date Published: November-2011
Source: Nature
Immune homeostasis in tissues is achieved through a delicate balance between pathogenic T-cell responses directed at tissue-specific antigens and the ability of the tissue to inhibit these responses. The mechanisms by which tissues and the immune system communicate to establish and maintain immune homeostasis are currently unknown. |
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Author: Pope J, Harding S, Khimdas S, et al
Date Published: January-2012
Source: The Journal of Rheumatology
We determined congruence with published guidelines from the European League Against Rheumatism (EULAR)/EULAR Scleroderma Trials and Research group, for systemic sclerosis (SSc) investigations and treatment practices within the Canadian Scleroderma Research Group (CSRG). |
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Author: Johnson SR, Granton JT, Tomlinson GA, et al
Date Published: January-2012
Source: The Journal of Rheumatology
Warfarin is recommended in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) and idiopathic PAH (IPAH) to improve survival. There is no evidence to support this in SSc-PAH and the evidence in IPAH is conflicting. We evaluated the ability of warfarin to improve survival using 2 large SSc-PAH and IPAH cohorts. |
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Author: Kathryn S. Torok and Thaschawee Arkachaisri
Date Published: January-2012
Source: The Journal of Rheumatology
To evaluate the effectiveness of a uniform single-center treatment protocol composed of high-dose methotrexate (MTX) and oral corticosteroids in a pediatric localized scleroderma (LS) cohort. |
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Author: Krzyszczak ME, Li Y, Ross SJ, et al
Date Published: October-2011
Source: Journal of Cellular and Molecular Medicine
Autoantibodies to topoisomerase I (topo I), RNA polymerase III (RNAPIII), centromere, U3RNP/fibrillarin, Th, PM-Scl, and U1RNP found in scleroderma (SSc) are associated with unique clinical subsets. The effects of race and gender on autoantibody prevalence and clinical manifestations were examined. |
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Author: Denton CP, Krieg T, Guillevin L et al
Date Published: January-2012
Source: Annals of the Rheumatic Diseases
The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). |
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Author: Capobianco J, Grimberg A, Thompson BM, et al
Date Published: January-2012
Source: Radiographics
Collagen vascular diseases are a diverse group of immunologically mediated systemic disorders that often lead to thoracic changes. The collagen vascular diseases that most commonly involve the lung are rheumatoid arthritis,… |
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Author: L. Mouthon
Date Published: January-2012
Source: Nature Reviews Rheumatology
Findings from ongoing studies of imatinib in systemic sclerosis (SSc) were eagerly awaited in 2011, but results from these clinical trials have so far been disappointing. However, progress in the understanding of the mechanisms that underlie SSc pathogenesis could provide clues to novel targets for 2012 and beyond. |
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Author: Arcucci A, Ruocco MR, Amatruda N, et al
Date Published: December-2011
Source: Journal of Biological Regulators
Systemic sclerosis (SSc) is a chronic disease of connective tissue characterized by vascular damage, autoantibody production and extensive fibrosis of skin, skeletal muscles, vessels and visceral organs. |
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Author: Martín JE, Bossini-Castillo L, Martín J.
Date Published: January-2012
Source: Human Genetics
Systemic sclerosis (SSc) is a severe connective tissue disorder characterized by extensive fibrosis, vascular damage, and autoimmune events. During the last years, the number of genetic markers convincingly associated with SSc has exponentially increased. |
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