Scleroderma Medical News: January 2009

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January 2009

Regional diastolic function by tissue Doppler echocardiography in systemic sclerosis (SSc): correlation with clinical variables. The incidence of left ventricular (LV) diastolic dysfunction is increased in SSc, while systolic dysfunction is present in a small percentage of patients. Linear correlation was observed between the extent of the diastolic abnormalities and the duration of disease. Longitudinal myocardial systolic velocities were significantly reduced in patients with abnormal DTI (doppler tissue imaging). E. Rosato. (SpringerLink) Rheumatology International 12/30/2008. (Also see: Cardiac Involvement)

Potential biomarkers for detecting pulmonary arterial hypertension (PAH) in patients with systemic sclerosis. The elevation of the serum levels of endoglin and endothelin-1 could represent a useful tool as PAH biomarkers. Nevertheless, the diagnostic value of these markers needs to be determined by prospective studies. P. Coral-Alvarado. (SpringerLink) Rheumatology International 01/07/2009 (Also see: Pulmonary Hypertension)

Pulmonary hypertension in systemic sclerosis determines cardiac autonomic dysfunction assessed by heart rate turbulence (HRT). HRT assessment indicates a frequent impairment of cardiac autonomic nervous system in SSc patients. Moreover, older age in these patients and especially PH are independent significant predictors of cardiac autonomic dysfunction. Bienias P. (PubMed) Int J Cardiol. 2008 Dec 19. (Also see: Pulmonary Hypertension and Cardiac Involvement)

Cardiac magnetic resonance imaging (MRI) in systemic sclerosis (SSc): a cross-sectional observational study of 52 patients. MRI is a reliable and sensitive technique to diagnose heart involvement in SSc and to analyse its mechanisms, including its inflammatory, microvascular and fibrotic components. Compared to echocardiography, MRI appears to provide additional information by visualising myocardial fibrosis and inflammation. A-L Hachulla. Annals of Rheumatic Diseases. 3 December 2008. (Also see: Cardiac Involvement)

N-terminal brain natriuretic peptide (NT-proBNP) in systemic sclerosis: a new cornerstone of cardiovascular assessment? NT-proBNP reliably detected the presence of cardiac involvement and appears as a very useful marker to risk stratify patients presenting with SSc. Y. Allanore. Annals of Rheumatic Diseases., BMJ 3 December 2008 (Also see: Cardiac Involvement)

Gastro-Esophageal Reflux and Pulmonary Fibrosis (ILD) in Scleroderma (SSc): A Study Using pH-Impedance Monitoring. SSc patients with ILD have more severe reflux i.e. more reflux episodes and more reflux reaching the proximal esophagus. Savarino E. (PubMed) Am J Respir Crit Care Med. 2008 Dec 18. (Also see: GERD and Pulmonary Fibrosis)

Nodular morphea. In nodular or keloidal scleroderma, patients develop lesions that are clinically indistinguishable from a keloid; however, the histopathological findings are more variable. Kauer F. (PubMed) Dermatology. 2009;218(1):63-6. Epub 2008 Nov 13. (Also see: Morphea)

arGentis Receives Favorable Opinion for European Orphan Drug Designation. arGentis Pharmaceuticals, LLC announced today that the European Medicines Agency's(EMEA) Committee for Orphan Medicinal Products (COMP) adopted a positive opinion recommending the company’s product candidate ARG201 (native type 1 bovine collagen) for the treatment of diffuse systemic sclerosis, also known as systemic scleroderma (SSc) for designation as an orphan medicinal product to the European Commission. arGentis Pharmaceuticals. 01/06/09. (Also see: Scleroderma Treatments)

Fatigue: an overlooked determinant of physical function in scleroderma. Our findings indicate that fatigue is common in scleroderma and that pain and fatigue are significant determinants of physical functioning for patients with limited and diffuse disease subtypes. Sandusky SB. (PubMed) Rheumatology (Oxford). 2008 Dec 23. (Also see: Fatigue)

AIMSPRO in Established Diffuse Cutaneous Systemic Sclerosis. To study the safety and tolerability of a hyperimmune goat serum product (AIMSPRO) in the treatment of systemic sclerosis (SSc) through a period of 26 weeks of study participation. The secondary objective of the study is to assess the efficacy of AIMSPRO as a therapeutic agent for SSc using inter alia the SSc-HAQ questionnaire and the modified Rodnan skin score. ClinicalTrials.gov. (Also see: Clinical Trials)

Oral Cyclophosphamide (CYC) for Active Scleroderma Lung Disease (SSc-ILD): A Decision Analysis. CYC therapy for 1 y results in a small loss in quality of life years compared with no CYC for SSc-ILD. The lack of a beneficial impact on survival and the transience of CYC's impact on decline in pulmonary function drive this conclusion. D Khanna. (Sage Journals) Medical Decision Making, Vol. 28, No. 6, 926-937 (2008). (Also see: Pulmonary Fibrosis)