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

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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