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This Article Full Text Full Text (PDF) All Versions of this Article: 34/2/371    most recent 09031936.00106008v2 09031936.00106008v1 Alert me when this article is cited Alert me if a correction is posted Permissions Request Permissions Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Google Scholar Articles by Overbeek, M. J. Articles by Grünberg, K. PubMed PubMed Citation Articles by Overbeek, M. J. Articles by Grünberg, K.

Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy

Depts of ¹ Pulmonary Diseases, ³ Rheumatology, and ⁴ Pathology; VU University Medical Center, Amsterdam, Depts of ² Rheumatology, and ⁵ Pulmonary Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.

CORRESPONDENCE: M. J. Overbeek, Dept of Pulmonary Diseases, VU University Medical Center, De Boelelaan 1117, P.O. Box 7057, 1007 MB, Amsterdam, The Netherlands. E-mail: mj.overbeek{at}VUmc.nl

Keywords: Intimal fibrosis, plexiform lesion, pulmonary arterial hypertension, pulmonary veno-occlusive disease, systemic sclerosis

Received: July 14, 2008
Accepted March 5, 2009

Systemic sclerosis-associated pulmonary arterial hypertension (SScPAH) has a worse prognosis and response to pulmonary arterial hypertension (PAH) therapy than idiopathic PAH (IPAH). These differences have not yet been explained. Knowledge concerning histological pulmonary vasculopathy in SScPAH is limited in contrast to IPAH. Therefore, we explored patterns of vasculopathy in SScPAH compared with IPAH.

Parameters of vasculopathy were assessed from lung tissue of eight PAH patients with limited cutaneous systemic sclerosis and 11 IPAH patients. Lung tissue was obtained at autopsy (n = 15), explantation (n = 3) and biopsy (n = 1).

Pulmonary arterial/arteriolar intimal fibrosis was identified in all SScPAH patients and in three IPAH patients (p = 0.003). Fibrosis of pulmonary veins/venules was found in all SScPAH patients and in three IPAH patients (p = 0.003). In four SScPAH patients, fibrosis of veins/venules was focal and associated with capillary congestion as in pulmonary veno-occlusive disease (PVOD). Of the IPAH patients, 10 had unequivocal evidence of plexogenic arteriopathy compared with none of the SScPAH patients (p = 0.001).

SScPAH is characterised by small vessel intimal fibrosis, which is associated with a PVOD-like pattern in some cases. This might explain its different clinical behaviour from IPAH. Small vessel intimal fibrosis may provide clues to elucidation of differences in pathogenetic mechanisms between the groups.

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