Scleroderma Medical News: December 2007

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December 2007

Reduced exercise capacity in systemic sclerosis (SSc) patients without pulmonary involvement. SSc patients without pulmonary impairment have reduced exercise capacity. Abnormal vascular response to exercise may account for this finding, as the vascular system is one of the major target organs in this pathological condition. Scandinavian Journal of Rheumatology, Volume 36, Issue 6 2007, pages 458 - 461. (InformaWorld) (Also see: Fatigue and Vasculitis )

Severe Fibrosis and Increased Expression of Fibrogenic Cytokines in the Gastric Wall of Systemic Sclerosis Patients. This study provides the first evidence that a severe fibrosis with a pronounced deposition of different collagen types, myofibroblast differentiation and an increased expression of several profibrotic factors are important hallmarks in the gastric wall of SSc patients. Mirko Manetti. 1217/449. ACR 2007. (Also see: GI Involvement )

Survey of More than 600 Patients Treated with Tracleer(R) (bosentan) Reveals Actelion Sure Steps(R) Patient Support Program Provides Valuable Educational Information. A recent Harris Interactive(R) survey shows that an overwhelming majority of Tracleer patients enrolled in Sure Steps find the patient support program valuable, and feel that their knowledge of pulmonary arterial hypertension (PAH) and Tracleer have improved due to the program. Bio-Medicine. 12/18/07. (Also see: Bosentan/Tracleer and SureSteps )

Chronic Articular (Joint) Involvement In Systemic Sclerosis. Articular disease appears in almost half of SSc patients in our series. The predominant clinical pattern is similar to that of rheumatoid arthritis, with bilateral and symetrical hand involvement. but with much less severe radiological damage and no association with rheumatoid factor (RF). Beatriz E. Joven. 7/7. ACR 2007. (Also see: Skeletal Involvement )

A Multi-Center Placebo-Controlled “In-Life” Study of MQX-503 in Patients with Raynaud Phenomenon (RP). MQX-503 (a novel formulation of topical nitroglycerin gel) is well-tolerated and more effective than placebo in the treatment of RP, with more pronounced effects during the winter season and in patients with primary disease. L. Chung. 2163/21. ACR 2007. (Also see: Raynaud's )

Early Atherosclerosis in Systemic Sclerosis (SSc) and Its Relation to Disease or Traditional Risk Factors. SSc is not associated with increased early signs of atherosclerosis. Martha E. Hettema. 1196/428. ACR 2007. (Also see: Cardiac Involvement )

Systemic Sclerosis and Systemic Lupus Erythematosus have Distinct Gene Expression Profiles in the Peripheral Blood Cells. These data show that a limited number of genes reliably distinguished SSc from SLE. Shervin Assassi. 38/38. ACR 2007. (Also see: Lupus and Scleroderma in Overlap )

Time to Diagnosis in Systemic Sclerosis: Is Gender a Factor? In SSc, the time to diagnosis is long, and it is longer for women than men. It is possible that there are differences in early disease progression between men and women with SSc, although there is currently no evidence for this. Marie Hudson 1201/433. ACR 2007. (Also see Difficult Diagnosis )

Familial Clustering of Auto-immune Diseases in Patients with Systemic Sclerosis. The high prevalence of other auto-immune diseases in families of SSc patients strongly suggests that there may be one shared gene or group of genes that predisposes to multiple autoimmune diseases. Marie Hudson. 1202/434. ACR 2007. (Also see: Causes of Scleroderma: Genetics )

Cardiac Involvement in Systemic Sclerosis: The Strongest Predictive Factor of Prognosis in Patients with Scleroderma. The results disclosed that most frequent cardiac manifestation at the initial evaluation of scleroderma is subclinical arrhythmia. More importantly, cardiac involvement at early stage of the disease is the strongest predictive factor for death. Sumiaki Tanaka. 13/13. ACR 2007. (Also see: Cardiac Involvement and Mortality and Prognosis )

Skin Thickness Progression Rate (STPR) in Systemic Sclerosis with Diffuse Cutaneous Involvement: A Predictor of Outcome. Rapid STPR at first evaluation in early dcSSc patients is a predictor of both internal organ involvement at one year after onset of skin thickening and 5 year mortality. Assessment of individual risk in dcSSc patients and planning of clinical trials involving these patients should include evaluation of STPR. (Also see: Mortality and Prognosis, Diffuse SSc, and Skin Fibrosis ).

Gastric Antral Vascular Ectasia (GAVE) in Patients with Systemic Sclerosis (SSc): a Systematic Review of the Literature. Although GAVE is a rare clinical entity, it should be considered in patients with SSc with iron-deficiency anemia. GAVE affects people with limited and diffuse SSc in equal proportions. Lisa Davis. 1194.426. ACR 2007. (Also see: GAVE )

Gastric Antral Vascular Ectasia (GAVE) in Systemic Sclerosis. Nearly 75% of the patients were on PPIs at the onset of GAVE, and it is interesting that prolonged use of these medications has been linked to the development of atrophic gastritis, another disease associated with GAVE. New anemia, particularly in early dcSSc patients, should prompt the clinician to carefully evaluate for GAVE. Kristin M. Ingraham. 15/15. ACR 2007. (Also see: GAVE )

Development of a Provisional Core Set of Response Measures for Clinical Trials of Systemic Sclerosis. Using Delphi exercise, we developed a provisional core set of measures for assessment of disease activity and severity in clinical trials of SSc. Dinesh Khanna. 2162. ACR 2007. (Also see: Clinical Trials )

Elevated Plasma Adrenomedullin (ADM) and Vascular Manifestations in Patients with Systemic Sclerosis (SSc). Patients who had both severe Raynaud's Phenomenon (RP) and elevated pulmonary arterial pressure were found to have significantly higher ADM levels than patients who had neither manifestations and those with severe RP alone. Elevated ADM was found in SSc patients with increased pulmonary arterial pressure regardless of concomitant interstitial lung disease. J Rheumatol 2007;34:2224-9. (Also see: Raynaud's and Pulmonary Hypertension )