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January 2014 Scleroderma News

Treatment of diffuse systemic sclerosis (dSSc) with hyperimmune caprine serum (AIMSPRO): a phase II double-blind placebo-controlled trial. These results confirm tolerability and safety of this novel biological agent in established dSSc and the value of a placebo treated control group in small clinical trials evaluating skin disease in SSc is confirmed. PubMed, Ann Rheum Dis, 2014 Jan 1;73(1):56-61. (Also see AIMSPRO Clinical Trial, and Diffuse Scleroderma)

Evidence for oesophageal and anorectal involvement in very early systemic sclerosis (VEDOSS). In patients with VEDOSS, oesophageal and anorectal disorders are frequently detected, showing that very early SSc is characterised by GI involvement. PubMed, Ann Rheum Dis, 2013 Oct 15. (Also see Gastrointestinal Involvement)

Nailfold capillary abnormalities in erectile dysfunction (ED) of systemic sclerosis (SSc). Neither the presence or absence of abnormal capillaroscopy findings nor the subdivision into early, active and late patterns is associated with coexistent ED in SSc. PubMed,Rheumatology, 2013 Dec 5. (Also see Nailfold Capillaroscopy for Scleroderma, and Erectile Dysfunction and Scleroderma)

Prevalence, Correlates and Outcomes of Gastric Antral Vascular Ectasia (GAVE) in Systemic Sclerosis. GAVE is rare and associated with a vascular phenotype, including anti-RNA-polymerase III antibodies, and a high risk of renal crisis. Anemia, usually requiring blood transfusions, is a common complication. Journal of Rheumatology, 12/01/2013. (Also see Gastric Antral Vascular Ectasia, Renal Involvement, and Anemia of Chronic Disease)

Key roles for interferon- and TGF-beta-regulated genes, and macrophage activation in progressive lung fibrosis associated with Systemic Sclerosis. This highlights major pathogenic pathways relevant to progressive pulmonary fibrosis in SSc-ILD: macrophage activation, and upregulation of TGF-beta- and IFN-regulated genes. PubMed, Arthritis Rheum, 2013 Nov 27. (Also see Pulmonary Fibrosis)

Increased risk for bone loss in women with systemic sclerosis: a comparative study with rheumatoid arthritis. There is an increased risk for bone loss in patients with SSc and the risk of osteoporosis is associated with disease severity, prolonged menopause and disease duration. PubMed, Int J Rheum Dis, 2013 Nov 28. (Also see Osteoporosis in Scleroderma)

Systemic sclerosis and the heart. Although no specific therapy exists for scleroderma heart disease, early recognition of the presence and type of scleroderma heart disease may lead to more effective management of patients. PubMed, Rheum Dis Clin North Am, 2014 Feb;40(1):87-102. (Also see Cardiac (Heart) Involvement in Systemic Sclerosis)

The inclusion of N-terminal pro-brain natriuretic peptide(NT-proBNP) in a sensitive screening strategy for systemic sclerosis-related pulmonary arterial hypertension(SSC-PAH): a cohort study. The combination of NT-proBNP with pulmonary function tests is a sensitive, yet simple and non-invasive, screening strategy for SSc-PAH. PubMed, Arthritis Res Ther, 2013 Nov 19;15(6):R193. (Also see Pulmonary Hypertension)

Systemic sclerosis, birth order and parity. Parity, age at first pregnancy and the gender of the first child are not relevant factors in our understanding of the epidemiology and pathogenesis of SSc. PubMed, Int J Rheum Dis, 2013 Nov 29. (Also see Causes of Scleroderma: Genetics)

Clinical Characteristics of Scleroderma Patients With and Without Pulmonary Hypertension. Scleroderma patients with pulmonary hypertension also present with more severe respiratory restriction and more impaired exercise capacity. Chest, 2013. (Also see Pulmonary Hypertension Diagnosis)

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