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Possible role of imatinib in clinical pulmonary veno-occlusive disease

Depts of ¹ Pulmonary Diseases, and ² Physiology, VU University Medical Center, Amsterdam, The Netherlands.

CORRESPONDENCE: A. Vonk-Noordegraaf, Dept of Pulmonary Diseases, VU University Medical Center, De Boelelaan 1117, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands. Fax: 31 204444328. E-mail: A.Vonk{at}vumc.nl

Keywords: Imatinib, platelet-derived growth factor receptor, pulmonary arterial hypertension, pulmonary veno-occlusive disease, vascular permeability

Received: May 6, 2007
Accepted December 10, 2007

The platelet-derived growth factor receptor inhibitor imatinib has demonstrated clinical and haemodynamical improvement in both animal models of pulmonary hypertension (PH) and patients with PH. It has been suggested that anti-proliferative effects on pulmonary vascular smooth muscle cells are responsible for these beneficial effects.

The current study describes a patient with pulmonary arterial hypertension associated with a suspected pulmonary veno-occlusive disease.

Treatment with imatinib resulted in rapid clinical improvement and decrease of ground-glass opacities and lobular septal thickening on high-resolution computed tomography.

Based on these findings and on in vitro effects of imatinib on permeability of the endothelium, the authors hypothesise that the rapid clinical outcome is partly due to effects of imatinib on vascular integrity.