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Risk factors for death and the 3-year survival of patients with systemic sclerosis: The French ItinérAIR-Sclérodermie study
Rheumatology, 02/23/09
Hachulla E et al. – This 3-year study reports that pulmonary arterial hypertension (PAH) increased the hypertension (HR) for mortality in patients with systemic sclerosis (SSc), justifying yearly echocardiographic screening; data consistent with previous observations.
Methods- Aim was to investigate the survival, risk factors, and causes of death in cohort of SSc pts w/o severe pulmonary fibrosis or severe left heart disease at baseline
- At 3-yr f/u, vital status was obtained from investigators or French national death records
- Causes of death were classified as SSc-related or otherwise
- Data were censored at 37 mo, time of death or loss to f/u, whichever was earlier
- 546 pts were followed for a median duration of 37 mo, representing 1547 patient-years
- At baseline, the majority of pts were female, with lcSSc, mean age 54.9 yrs and mean duration of SSc of 8.8 yrs
- 47 pts died, giving a 3-yr survival of 91.1% and cumulative mortality of 3.04 deaths/100 patient-yrs
- 32.2% deaths resulted from PAH, and 17.1% from cancer
- 20/47 pts with PAH at baseline died during f/u, giving a 3-yr survival of 56.3%
- Multivariate analysis: PAH, age at first symptom, duration of SSc, and Rodnan skin score were associated with increased mortality
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Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil
Rheumatology, 12/18/09
Rheumatic heart disease and its control in the Pacific
Expert Review of Cardiovascular Therapy , 12/15/09
Heart rate turbulence assessment in systemic sclerosis: The role for the detection of cardiac autonomic nervous system dysfunction
Rheumatology, 12/11/09
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