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August 2013 Scleroderma News

Increased expression of S100A8 and S100A9 in patients with diffuse cutaneous systemic sclerosis (dcSSc). A correlation with organ involvement and immunological abnormalities. These two proteins may play important roles in the development of systemic sclerosis. PubMed, Clin Rheumatol, 2013 Jun 11. (Also see Causes of Scleroderma: Proteins)

Bosentan and sildenafil: successful treatment in a sclerodermic patient with refractory ulcers. Treatment resulted in complete healing of old ulcers both to upper and lower limbs and allowed the interruption of intravenous therapies. Reumatisimo, 2013-05-27. (Also see Bosentan/Tracleer and Sildenafil/Viagra)

Treating skin and lung fibrosis in systemic sclerosis: a future filled with promise? The most promising targets include inhibitors of B-cells, tyrosine kinases, 5-hydroxytryptamin receptors, interleukin-6 and Wnt signalling. PubMed, Curr Opin Pharmacol, 2013 Jun;13(3):455-62. (Also see Skin Fibrosis, and Pulmonary Fibrosis Research)

Survival and prognostic factors in systemic sclerosis (SSc)-associated pulmonary hypertension (PAH). This analysis confirms a poor pooled 3-year survival rate of 52% (47-58) in SSc-associated PH. PubMed, Arthritis Rheum, 2013 Jun 5. (Also see Pulmonary Hypertension Prognosis)

Increased circulating soluble vascular adhesion protein-1(sVAP-1) levels in systemic sclerosis (SSc): association with lower frequency and severity of interstitial lung disease. Study results suggest that sVAP-1 plays a role in the pathogenesis of interstitial lung disease in SSc. International Journal of Rheumatic Diseases, 28 May 2013. (Also see Pulmonary Fibrosis Research)

Case Report: Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease (MCTD) resulting in dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition. A case of MCTD consisting of scleroderma, Sjögren's syndrome and polymyositis complicated by scleroderma renal crisis (SRC) but with negative anti-nuclear antibody (ANA), anti-Scl 70 and anti-centromere antibodies. Springer Link, May 2013;58:153 (Also see Scleroderma Renal Crisis, Mixed Connective Tissue Disease)

Prevalence of pulmonary arterial hypertension (PAH) in patients with connective tissue diseases: a systematic review of the literature. Prevalence of PAH in patients with connective tissue diseases was substantially higher than that of idiopathic PAH based on pooled prevalence estimates. PubMed, Clin Rheumatol, 2013 Jun 20. (Also see Pulmonary Hypertension Research and Connective Tissue Disease)

High serum sCD163/sTWEAK ratio is associated with lower risk of digital ulcers but more severe skin disease in patients with systemic sclerosis (SSc). Further studies are required to reveal whether targeting of the CD163-TWEAK pathway might be a potential strategy for treating vascular disease and/or skin fibrosis in SSc. Arthritis Research & Therapy, June 24 2013, 15:R69. (Also see Research on Digital Ulcers)

Survival in Systemic Sclerosis with Pulmonary Arterial Hypertension Has Not Improved in the Modern Era. Despite an improvement in clinical status, unlike patients with pulmonary arterial hypertension, the mortality in systemic sclerosis has not improved since the introduction of epoprostenol. CHEST Journal, June 6 2013. (Also see Pulmonary Hypertension Prognosis and Flolan/Prostacyclin/Epoprostenol)

Go to Scleroderma Medical News: July 2013
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