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June 2014 Scleroderma News

Solitary organizing pneumonia (SOP) in systemic sclerosis (SSc) mimicking lung adenocarcinoma. SOP is often misdiagnosed and removed surgically as it is rare and difficult to distinguish from lung carcinoma. SOP should be considered when a solitary nodular lung lesion is noted in a patient with SSc. PubMed, Arthritis Rheumatol, 2014 Apr 22. (Also see Lung Involvement: Pneumonia and Cancer and Scleroderma)

Echocardiographic follow-up of patients with systemic sclerosis (SSc) by 2D speckle tracking echocardiography (STE) of the left ventricle (LV). STE might be a sensititive and valuable method to detect early LV systolic impairment in patients with SSc and preserved LV ejection fraction during two years and prospective evaluations are needed for prognostic implications of these changes. PubMed, Cardiovasc Ultrasound, 2014 Mar 29;12(1):13. (Also see Scleroderma Heart Involvement)

Improved transplant-free survival in patients with systemic sclerosis-associated pulmonary hypertension and interstitial lung disease (SSc-PH-ILD). Survival of patients with SSc-PH-ILD has modestly improved, and may partly be due to aggressive PAH-targeted therapy. PubMed, Arthritis Rheumatol, 2014 Apr 11. (Also see Lung Transplant for Pulmonary Fibrosis)

Five-year follow-up of left ventricular diastolic function in systemic sclerosis (SSc) patients: determinants of mortality and disease progression. In SSc patients left ventricular diastolic dysfunction is highly prevalent and is associated with increased risk of mortality and the data suggests that in the advanced phase of the disease the myocardial fibrotic processes burns out while the increase of the filling pressure progresses continuously. Seminars in Arthritis and Rheumatism, 11 April 2014. (Also see Scleroderma Heart Involvement)

Impact of anti-centromere antibodies (ACA) on pulmonary function test (PFT) results in patients with systemic sclerosis without established or suspected pulmonary disease. Patients with ACA, without established or suspected pulmonary complications, have PFT abnormalities consistent with indolent increased pulmonary vascular resistance despite the majority of such patients not subsequently developing PAH. PubMed, Clin Rheumatol, 2014 Apr 22. (Also see Scleroderma Autoantibodies and Pulmonary Hypertension Diagnosis)

Systemic sclerosis sine scleroderma (ssSSc) and limited cutaneous systemic sclerosis (lcSSc): Similarities and differences. ssSSc and lcSSc patients share demographic, clinical and immunologic features although differences are mainly due to peripheral vascular manifestations. ssSSc patients should be truly differentiated from early SSc using sensitive and specific studies looking for any asymptomatic organ involvement. PubMed, Clin Exp Rheumatol, 2014 Apr 28. (Also see Systemic Sclerosis Sine Scleroderma and Limited Scleroderma)

Fibrosis is a lethal component of systemic sclerosis. The chemical and mechanical signals that drive fibroblast activation and myofibroblast differentiation are discussed, which could serve as targets for new therapies for fibrosis in SSc. PubMed, Nat Rev Rheumatol, 2014 Apr 22. (Also see: Skin Fibrosis and Causes of Scleroderma: Fibroblasts)

MRI Helps ID Heart Risk in Scleroderma. Cardiac MRI could help provide diagnostic information about potential arrhythmogenic events among patients with scleroderma being considered for implantable cardioverter defibrillators (ICDs). Med Page Today, May 6 2014. (Also see Heart Involvement)

Systemic sclerosis (SSc): comparison of efficacy of oral cyclophosphamide (CYC) and azathioprine (AZA) on skin score and pulmonary involvement-a retrospective study. The aim of this study was to evaluate efficacy of (AZA) and (CYC) as a therapeutic regimen for interstitial lung disease associated with (SSc) and the results indicated that AZA can be effective in ameliorating or stabilizing lung function in selected SSc patient groups. PubMed, Rheumatol Int, 2014 May 7. (Also see Pulmonary Fibrosis and Immunosuppressants)

Disease-related nutritional risk and mortality in systemic sclerosis (SSc). The combined assessment of nutritional parameters and disease activity significantly improves the evaluation of mortality risk and disease-related nutritional risk screening should be systematically included in the clinical workup of every SSc patient. PubMed, Clin Nutr, 2014 Jun;33(3):558-61. (Also see: Diet and Systemic Scleroderma)

Go to Scleroderma Medical News: May 2014
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