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December 2016 Scleroderma News

Prevalence of Systemic Sclerosis (SSc) in Primary Biliary Cholangitis (PBC) Using the New American College of Rheumatology (ACR)/EULAR Classification Criteria. Our data show a high prevalence of SSc in patients with PBC with probable underestimation by previous studies using the original ACR criteria. PubMed, J Rheumatol, 10/15/2016. (Also see Liver and Spleen Involvement)

Mesenchymal stem cell therapy for refractory scleroderma: a report of 2 cases. Stem cell therapy is not ready for use on every patient with scleroderma but there are reasons to believe that it is possibly effective where there is indeed no hope to offer. PMC, Stem Cell Investig, 09/19/2016. (Also see Stem Cell (Bone Marrow) Transplantation)

Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis (SSc): A Longitudinal EUSTAR Study. In SSc patients presenting early after Raynaud's phenomenon onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. PubMed, PLoS One, 2016 Oct 5;11(10):e0163894. (Also see Raynaud's)

Mycophenolate mofetil for scleroderma–related interstitial lung disease. Although this study does not provide evidence for equivalency to cyclophosphamide, its clear benefit in patient tolerability made MMF the preferred initial treatment in an arena with limited options. The Lancet Respiratory Medicine, 2016 Nov;4(11):e53. (Also see Pulmonary Fibrosis Treatments: Cellcept)

Cytori Presents Scleroderma Clinical Trial Data at the 2016 American College of Rheumatology Annual Meeting. Cytori Therapeutics, Inc. present data from its STAR Phase 3 clinical trial describing progress, preliminary blinded patient characteristics, and safety of adipose–derived regenerative cells (ADRCs) for treatment of scleroderma hand dysfunction at the Annual Meeting of the American College of Rheumatology on November 15, 2016. Business Wire, 10/26/2016. (Also see Scleroderma Clinical Trials)

Bosentan and macitentan (ERA's) prevent the endothelial–to–mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study. The present study provides further in vitro evidence of the use of ERA in inhibiting the EndoMT process, supporting the clinical efficacy of these drugs in SSc therapy and their usefulness for interfering with progressive fibrosis. PMC, Arthritis Res Ther, October 2016; 18: 228. (Also see Tracleer (Bosentan), Macitentan (Opsumit™) and Fibroblasts)

Clinical relevance of antiphospholipid antibodies in systemic sclerosis (SSc): A systematic review & meta–analysis. A strong relationship exists between anticardiolipin antibodies and aß2GPI of IgG/IgM isotype and SSc; patients positive for these antibodies are more likely to suffer from pulmonary arterial hypertension, renal disease, thrombosis and digital infarction/ischaemia. Seminars in Arthritis and Rheumatism, 11/05/2016. (Also see Antiphospholipid Syndrome)

A multi–center, open–label, comparative study of B–cell depletion therapy with Rituximab (RTX) for systemic sclerosis–associated interstitial lung disease. Our data indicate that RTX has a beneficial effect on lung function and skin fibrosis in patients with SSc and randomized controlled studies are highly needed. Seminars in Arthritis and Rheumatism, 11/05/2016. (Also see Biologic Agents)

Pharyngoesophageal dysphagia: an under recognised, potentially fatal, but very treatable feature of systemic sclerosis (SSc). Pharyngoesophageal dysphagia in SSc, is a rare, very distressing and potentially fatal manifestation that can have a very favourable outcome with appropriate management. PubMed, Intern Med J, 2016 Nov;46(11):1340-1344. (Also see Dysphagia)

Transforming growth factor beta (TGF–beta1), int/Wingless (WNT) and sonic hedgehog (SHH) signaling in tumor progression and in fibrotic diseases. In this review, we focus on TGF–beta, WNT and SHH signal transduction pathways and describe small molecule inhibitors that are used in phase I/II clinical trials to treat fibrosis or fibrotic cancers. PubMed, Front Biosci (Schol Ed), 2017 Jan 1;9:31-45. (Also see Fibroblasts)

Go to Scleroderma Medical News: November 2016

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