%0 Journal Article %A Stephen Mathai %A Kaitlin Kroening %A Laura Hummers %A Ari Zaiman %A Reda Girgis %A Paul Hassoun %T Differences between limited and diffuse systemic sclerosis-related pulmonary arterial hypertension %D 2011 %J European Respiratory Journal %P p2331 %V 38 %N Suppl 55 %X Background: Pulmonary arterial hypertension (PAH) is a leading cause of death in systemic sclerosis (SSc). Frequently associated with t limited SSc (lcSSc), PAH can also complicate diffuse SSc (dcSSc). Little is known about the characteristics of patients with dcSSc-PAH compared to lcSSc-PAH.Methods: Consecutive patients with SSc-PAH were followed prospectively. LcSSc and dcSSc were defined according to the American College of Rheumatology criteria. PAH was defined as a mPAP> 25mmHg with a PCWP ≤ 15 mmHg in the absence of significant ILD. Survival was assessed from time of diagnosis of PAH.Results: 133 with SSc-PAH were included; 112 (84%) had lcSSc. LcSSc were more likely to be women (92% vs. 71%, p<0.01). DcSSc patients had a higher serum creatinine (1.0±0.4 mg/dL vs. 1.3±0.7, p=0.01) a higher proportion of hyponatremia (33% vs. 15%, p=0.05), and a shorter 6MWD (264±101 vs. 357±119m, p=0.02), but there were no significant differences in PFTs. Hemodynamics revealed less severe PAH in dcSSc with a lower mPAP (38±8 vs. 43±13mmHg, p=0.08) and PVR (6.8±3.6 vs. 9.4±6.2 Wood units, p=0.07). However, there was no difference in survival between the groups.Conclusions: In this cohort of SSc-PAH patients, 15% of subjects had dcSSc. While many characteristics were similar between the lcSSc and dcSSc groups, dcSSc patients were more likely to be men, have hyponatremia, renal insufficiency, and shorter 6MWD. Still, even though hemodynamics suggested less severe disease in dcSSc, survival did not differ between lcSSc and dcSSc. Whether this reflects intrinsic differences in the response to PAH between lcSSc and dcSSc remains to be determined and should be the focus of future investigation. %U