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Is pulmonary arterial hypertension really a late complication of systemic sclerosis
Chest, 10/12/09

Hachulla E et al. . –In contrast to the expected scenario, early onset PAH occurred in approximately half of SSc patients. Early onset PAH was as frequent among patients with diffuse as limited SSc. Annual screening for PAH should be implemented immediately after SSc diagnosis for all patients.

Methods

Clinical and hemodynamic data at time of diagnosis collected retrospectively
78 consecutive patients with SSc associated PAH
PAH diagnosed within 5 years of SSc considered early onset complication
PAH diagnosed > 5 years following SSc diagnosis considered a late complication

Results

PAH occurred 6.3 ± 6.6 years after diagnosis of SSc (median delay 4.0 years)
Early onset PAH diagnosed in 43 patients (55.1%) and late onset PAH in 35 patients (44.9%)
Patients with early onset PAH older at SSc diagnosis than patients with late onset (58.0 ± 12.5 vs 46.6 ± 12.9 years)
No differences in age at time of PAH diagnosis, or SSc subtype (limited vs diffuse; anticentromere vs anti-Scl70 antibodies), observed between onset subgroups
At diagnosis, early onset PAH more severe than late onset, with lower cardiac index (2.4 ± 0.6 vs 2.8 ± 0.6 L/min/m2) and greater total pulmonary resistance (1,708 ± 777 vs 1,341 ± 530 dyn|b1sec/cm5/m2)
Mortality at 3 and 5 years comparable between subgroups

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Pulmonary hypertension in pregnancy: two cases and review of the literature
Internal Medicine Journal, 11/11/09 Relevance Score: 87%

Bosentan: A Review of its Use in the Management of Mildly Symptomatic Pulmonary Arterial Hypertension
American Journal of Cardiovascular Drugs, 10/08/09 Relevance Score: 85%

Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis
Internal Medicine Journal, 10/21/09 Relevance Score: 84%

Right Ventricular Free Wall Pacing Improves Cardiac Pump Function in Severe Pulmonary Arterial Hypertension: a Computer Simulation Analysis
Heart And Circulatory Physiology, 10/20/09 Relevance Score: 84%

Measures of Response in Clinical Trials of Systemic Sclerosis: The Combined Response Index for Systemic Sclerosis (CRISS) and Outcome Measures in Pulmonary Arterial Hypertension Related to Systemic Sclerosis (EPOSS)
Journal of Rheumatology, 10/15/09 Relevance Score: 84%