PT - JOURNAL ARTICLE AU - HERMAN M.A. HOFSTEE AU - ALEXANDRE E. VOSKUYL AU - ANTON VONK NOORDEGRAAF AU - YVO M. SMULDERS AU - PIET E. POSTMUS AU - BEN A.C. DIJKMANS AU - ERIK H. SERNÉ TI - Pulmonary Arterial Hypertension in Systemic Sclerosis Is Associated with Profound Impairment of Microvascular Endothelium-dependent Vasodilatation AID - 10.3899/jrheum.110397 DP - 2012 Jan 01 TA - The Journal of Rheumatology PG - 100--105 VI - 39 IP - 1 4099 - http://www.jrheum.org/content/39/1/100.short 4100 - http://www.jrheum.org/content/39/1/100.full SO - J Rheumatol2012 Jan 01; 39 AB - Objective. Impaired microvascular function may contribute to organ complications in patients with systemic sclerosis (SSc). We investigated whether SSc patients with and without pulmonary arterial hypertension (PAH) show a graded impairment of microvascular function compared to healthy controls. Methods. Twenty-two patients with SSc and 22 controls were studied. All patients underwent right heart catheterization; 6 had no PAH (SSc-nonPAH) and 16 had PAH (SSc-PAH). Acetylcholine (ACh)-mediated endothelium-dependent vasodilatation and sodium nitroprusside (SNP)-mediated endothelium-independent vasodilatation were assessed by iontophoresis combined with laser Doppler flowmetry. Results. Compared to sex- and age-matched controls, ACh-mediated vasodilatation was reduced in SSc-PAH (340.4% vs 79.5%, respectively; p < 0.01), but not in SSc-nonPAH (340.4% vs 397.9%; p = 0.90). No significant differences were present between the groups in SNP-mediated vasodilatation. Conclusion. Systemic microvascular endothelium-dependent vasodilatation is markedly reduced in SSc complicated by PAH.