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February 2018 Scleroderma News

Myeloablative Autologous Stem–Cell Transplantation for Severe Scleroderma. Myeloablative autologous hematopoietic stem–cell transplantation achieved long–term benefits in patients with scleroderma, including improved event–free and overall survival, at a cost of increased expected toxicity. PubMed, N Engl J Med, 2018 Jan 4;378(1):35-47. (Also see Stem Cell Transplantation)

Impact of Radiation Therapy on Scleroderma and Cancer Outcomes in Scleroderma Patients with Breast Cancer. These data suggest that radiation injury causing local tissue fibrosis is not inevitable in SSc patients with breast cancer, occurring in 50% of cases without evidence of lung or generalized skin disease flare. PubMed, Arthritis Care Res (Hoboken), 01/05/2018. (Also see Cancer and Radiation and Scleroderma)

Factors associated with the 6–minute walk distance (6MWD) in patients with systemic sclerosis (SSc). During SSc, the 6MWD is independently associated with initial HR and HR variation; suggesting that pulmonary vasculopathy may have a greater impact than parenchymal involvement on functional limitation. PubMed, Arthritis Res Ther, 2017 Dec 15;19(1):279. (Also see Pulmonary Fibrosis Diagnosis)

Do scleroderma patients look young?: Evaluation by using facial imaging system. The severity of wrinkles, texture and pores were significantly lower in SSc patients than control subjects and among them, wrinkles showed better correlation with skin thickness score. PubMed, Drug Discov Ther, 2017;11(6):342-345. (Also see Skin Fibrosis)

Skin mapping for the classification of generalized morphea. Symmetric and isomorphic subsets possess distinctive demographic and clinical features, suggesting they more accurately define the phenotype of generalized morphea. PubMed, J Am Acad Dermatol, 2018 Feb;78(2):351-357. (Also see Morphea)

Disease staging and sub setting of interstitial lung disease associated with systemic sclerosis (SSc–ILD): impact on therapy. In the management of SSc–ILD, a multidisciplinary team approach which integrates physiology and radiology with the patient at the centre of the process is crucial for effective management and provision of the best outcomes. PubMed, Expert Rev Clin Immunol, 01/10/2018. (Also see Treatments for Pulmonary Fibrosis)

Plasmacytoid dendritic cells (pDC) promote systemic sclerosis (SSc) with a key role for TLR8. We conclude that the pDC is an essential cell type involved in the pathogenesis of SSc and its removal using depleting antibodies or attenuating pDC function could be a novel approach to treat SSc patients. PubMed, Sci Transl Med, 2018 Jan 10;10(423). (Also see Dendritic Cells)

Cyclophosphamide for connective tissue disease–associated interstitial lung disease. Researchers may consider comparing cyclophosphamide versus antifibrotic agents, or comparing both versus placebo, in particular, for those with evidence of rapidly progressive fibrotic disease, who may benefit the most. PubMed, Cochrane Database Syst Rev, 01/03/2018. (Also see Treatments for Pulmonary Fibrosis: Oral and IV Cyclophosphamide)

Esophageal symptoms and their lack of association with high–resolution manometry (HRM) in systemic sclerosis patients. There was no clear association between symptomatology and HRM findings and HRM does not seem to accurately predict upper gastrointestinal symptomatology. PubMed, Reumatol Clin, 12/16/2017. (Also see Dysmotility Syndrome)

An evaluation of two novel capillaroscopy techniques in suspected scleroderma–spectrum disorders: A single–centre cross–sectional study. New technologies could help to improve accessibility to nailfold capillaroscopy, an important diagnostic tool and putative biomarker in scleroderma–spectrum disorders, whilst retaining accurate results. PubMed, Mod Rheumatol, 2017 Dec 20:1-5. (Also see Nailfold Capillaroscopy)

Go to Scleroderma Medical News: January 2018

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