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Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: A distinctive vasculopathy
European Respiratory Journal , 04/22/09

Overbeek MJ et al. – Study shows that systemic sclerosis-associated pulmonary arterial hypertension (SScPAH) is characterised by small vessel intimal fibrosis, which is associated with a pulmonary veno-occlusive disease (PVOD)-like pattern in some cases. This might explain its different clinical behaviour from idiopathic PAH.

Methods

Study aims to determine the histologic pulmonary vasculopathy in SScPAH compared with IPAH
Parameters of vasculopathy were assessed of lung tissue of 8 PAH pts with limited cutaneous SSc and 11 IPAH pts

Results

Pulmonary arterial/arteriolar intimal fibrosis was identified in all SScPAH and in 3/11 IPAH pts
Fibrosis of pulmonary veins/venules was found in all SScPAH and in 3/11 IPAH pts
In 4 SScPAH pts fibrosis of veins/venules was focal and associated with capillary congestion as in pulmonary veno-occlusive disease (PVOD)
10/11 IPAH pts had unequivocal evidence of plexogenic arteriopathy vs none of the SScPAH pts

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