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December 2019 Scleroderma News

Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. In patients with progressive fibrosing interstitial lung diseases, the annual rate of decline in the forced vital capacity was significantly lower among patients who received nintedanib than among those who received placebo. PubMed, N Engl J Med, 2019 Oct 31;381(18):1718-1727. (Also see Clinical Trials and Treatments for Pulmonary Fibrosis)

Clinical and laboratory predictions of myocardial inflammation as detected by cardiac magnetic resonance imaging in patients with systemic sclerosis: A pilot study. An increased risk of myocardial inflammation was associated with young age and high modified Rodnan skin score at onset. PubMed, Int J Rheum Dis, 10/29/2019. (Also see Cardiac (Heart) Involvement)

Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure (mPAP) associated with systemic sclerosis. This is the first randomized, double-blind, placebo-controlled study testing the effect of ambrisentan in patients with mildly elevated mPAP and/or exercise pulmonary hypertension. PubMed, Arthritis Res Ther, 2019 Oct 26;21(1):217. (Also see Letairis (Ambrisentan), Endothelin Receptor Antagonists and Clinical Trials)

LAUDES Study: impact of digital ulcers (DUs) on hand functional limitation, work productivity and daily activities, in systemic sclerosis (SSc) patients. Study findings show an impaired hand function and increased disability for daily life activities and work productivity in SSc patients with DUs compared with patients without DUs in Spanish population. PubMed, Rheumatol Int, 2019 Nov;39(11):1875-1882. (Also see Digital Ulcers)

Interferon Lambda (IFNL3) genotype is associated with pulmonary fibrosis (PF) in patients with systemic sclerosis (SSc). IFNL3 serum levels and the genetic variant known to be associated with liver fibrosis are similarly linked to PF, but not to worsening of skin fibrosis in SSc. PubMed, Sci Rep, 2019 Oct 16;9(1):14834. (Also see Pulmonary Fibrosis)

The role of high-resolution manometry in the assessment of upper gastrointestinal involvement in systemic sclerosis: a systematic review. Esophageal body dysmotility on HRM positively correlates with the presence of interstitial lung disease. PubMed, Clin Rheumatol, 11/11/2019. (Also see Dysmotility Syndrome and Correlation between Pulmonary Fibrosis and other Diseases)

Case Report: A case of concurrent systemic sclerosis and scleredema (SAB). This case highlights multiple disease associations of SAB and the importance of high clinical suspicion in atypical disease presentations. PMC, JAAD Case Rep, 2019 Nov; 5(11): 940–942. (Also see Scleredema Adultorum Buschke)

Genetic Interactions Affect Lung Function in Patients with Systemic Sclerosis. We identified a three-gene network comprising WNT5A, RBMS3 and MSI2, which in combination influenced multiple pulmonary pathology measures. PubMed, G3 (Bethesda), 11/06/2019. (Also see Genetics and Pulmonary Fibrosis Diagnosis)

Case Report: A case of overlap syndrome (scleroderma and polymyositis) associated with the development of sudden chest pain due to myocardial damage. Synthetic judgement of serum brain natriuretic peptide/ troponin T levels and cardiac MRI is useful in the search for the cause of chest symptoms even in patients with collagen diseases. PubMed, Drug Discov Ther, 2019;13(5):297-298. (Also see Cardiac (Heart) Involvement, Symptoms/Complications of Polymyositis and Scleroderma in Overlap)

Racial differences in systemic sclerosis (SSc) disease presentation: a European Scleroderma Trials and Research group study. We evaluated differences in SSc presentations between white patients, Asian patients and black patients and analysed the effects of geographical locations. PubMed, Rheumatology (Oxford), 11/04/2019. (Also see Ethnicity, Race and Geographical Regions)

Go to Scleroderma Medical News: November 2019
 

In Loving Memory of Lorraine Wempner, a donation to tackle scleroderma has been made by Paul Wempner. Posted 11/13/2019. (Also see: ISN News)

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