Scleroderma Medical News: October 2008

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October 2008

Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and a meta-analysis of randomized controlled trials and observational prospective cohort studies. Cyclophosphamide treatment in patients with systemic sclerosis related interstitial lung disease does not result in clinically significant improvement of pulmonary function. (UnBound) C. Nannini. Arthritis Res Ther 2008 Oct 20; 10(5):R124. (Also see: Cytoxan, Clinical Trials, and Pulmonary Fibrosis)

Asymmetrical dimethylarginine (ADAM) in systemic sclerosis-related pulmonary arterial hypertension (PAH). In patients with SScPAH, increased ADMA serum levels and their negative association with exercise capacity suggests that the nitric oxide pathway is involved in the development of pulmonary vascular disease. T. Dimitroulas. Rheumatology 2008 47(11):1682-1685. (Also see: Pulmonary Hypertension)

Connective Tissue Disease Associated Pulmonary Arterial Hypertension in the Modern Treatment Era. Survival in SSc-PAH in the modern treatment era is better than in historical series. A significant proportion of patients with mild functional impairment or exercise-induced SSc-PAH have evidence of disease progression. Survival in patients with respiratory disease associated pulmonary hypertension is inferior. R. Condliffe. Am. J. Respir. Crit. Care Med. October, 2008. (Also see: Connective Tissue Disease and PAH)

Working ability in relation to disease severity, everyday occupations and well-being in women with limited systemic sclerosis. Fifty per cent of the women were restricted in their working ability; the lower the working ability, the lower their perceived well-being. G. Sandqvist. Rheumatology 2008 47(11):1708-1711. (Also see: Quality of Life)

Digital ulcers and outcomes assessment in scleroderma.. Healing has been demonstrated with intravenous iloprost and two studies with bosentan have demonstrated reduction in the occurrence of new digital ulcers (DUs) over 4-6 months of treatment. (PubMed) Matucci-Cerinic M. Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v46-7. (Also see: Digital Ulcers and Bosentan)

Right ventricular function in scleroderma-related pulmonary hypertension (SSc-PAH) . Recent echocardiographic and haemodynamic studies suggest that right ventriculer (RV) pump function and filling characteristics are altered in SSc-PAH as compared with idiopathic PAH. (UnBound MedLine) Vonk Noordegraaf A. Rheumatology (Oxford) 2008 Oct.:v42-3. (Also see: Cadiac Involvement and PAH)

Clinical Trial
Open Enrollment!

Safety, Tolerability and Pharmcodynamic Profile of Oral 2101 Once Daily for up to 14 Days in Secondary Raynaud's Disease. To investigate the severity of secondary Raynaud's disease-related attachks during the 14 days 2101 dosing period. Currently Recruiting. University des Saarlandes. Homburg, Germany. ClinicalTrials.gov. (Also see: Raynaud's)

Biomarkers of vascular disease in scleroderma. Vascular disease is present in every patient with scleroderma and is a major source of morbidity and mortality. There is a subset of patients who will develop severe and sometimes life-threatening vascular events. (PubMed) Hummers LK. Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v21-2. (Also see: Digital Ulcers, and Pulmonary Hypertension)

Systemic Sclerosis Has Unique Cardiac Risk. Periodic cardiac evaluations should be routine for all patients with systemic sclerosis, starting at “the first day of the disease. Rheumatology News. Volume 7, Issue 9, Page 33 (September 2008). (Also see: Cardiac Involvement)

Proteomic analysis of scleroderma (SSc) lesional skin reveals activated wound healing phenotype of epidermal cell layer. We show that SSc epidermis has an activated, wound healing phenotype. These findings are important because epidermal cells activated by injury induce and regulate local fibroblasts during wound repair. (UnBound) Aden N. Rheumatology (Oxford) 2008 Oct 1. (Also see: Skin Involvement)

Histopathology and bronchoalveolar lavage. Studies employing lung biopsy material and BAL fluid (BALF) have provided insight into the pathogenesis of scleroderma-associated interstitial lung disease (SSc-ILD). (UnBound MedLine) Silver RM. Rheumatology (Oxford) 2008 Oct.:v62-4. (Also see: Pulmonary Fibrosis)

Vitamin D, Parathyroid Hormone, and Acroosteolysis in Systemic Sclerosis. Low levels of vitamin D may reflect silent malabsorption and might be a risk factor for secondary hyperparathyroidism and bone resorption. Y. Braun-Moscovici. J Rheumatol. Oct 1 2008. (Also see: Vitamin D Deficiency, Calcinosis, and Bone Resorption)

New Systemic Sclerosis Classification Created. One in five patients with clinical symptoms characteristic of systemic sclerosis do not fit into the popular classification scheme that divides the disease into the diffuse and limited cutaneous subtypes, according to Dr. Thomas Krieg. B. Jancin. Rheumatology News. Volume 7, Issue 9, Page 27 (September 2008) (Also see: Types of Scleroderma, Overlap, and Undifferentiated)

Clinical Trial
Open Enrollment!

Pulmonary Fibrosis with Systemic Sclerosis Open Enrollment. Novartis Pharmaceuticals. Reposted 10/01/08. (Also see: Scleroderma Clinical Trials)

Morphea Patient
Registry!

First U.S. Morphea Registry and DNA Repository for both Adults and Children Established. Southwestern Medical Center. Reposted 10/01/08. (Also see: Morphea and Research Registries )