Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement
The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.
An Analysis of Outcomes in Patients with Systemic Sclerosis- and Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension
Chung1, Lorinda, Liu1, Juliana, Parsons2, Lori, Hassoun3, Paul M., McGoon4, Michael, Badesch5, David, Miller2, Dave P.
Patients with systemic sclerosis associated pulmonary arterial hypertension (SSc-APAH) have extremely poor outcomes with high mortality rates. A previous study indicated that SSc-APAH is associated with poorer survival than systemic lupus erythematosus (SLE)-APAH. To gain insight regarding pathophysiologic mechanisms responsible for differences between these two groups, their presentation and outcomes were compared.
The Registry to Evaluate Early and Long Term PAH Management (REVEAL) is a prospective registry of >3,000 patients with World Health Organization Class I pulmonary hypertension from 54 US centers. Kaplan-Meier curves were estimated for survival, freedom from hospitalization, and freedom from initiation of parenteral PAH therapy in patients with SSc-APAH vs. SLE-APAH from the time of enrollment. Differences in outcomes between the groups were assessed by the log-rank test. Multivariable Cox proportional hazards models were developed to assess the risk for poorer outcomes in patients with SSc-APAH, controlling for B-type natriuretic peptide (BNP) levels, diffusing capacity of carbon monoxide (DLCO), pulmonary vascular resistance (PVR), and renal insufficiency (investigator clinical judgment).
Patients with SSc (302: 78 diffuse, 224 limited) had higher BNP levels than patients with SLE (105) with mean(SD) values of 471.2(889.5) vs. 288.0(349.4) pg/mL (p=0.03). DLCO was significantly lower in the SSc patients (43.2(16.6) vs. 52.6(19.1) % predicted, p=0.0003). Baseline mean pulmonary artery pressure and PVR were lower in the SSc group, (44.0(11.9) vs. 46.7(9.2) mmHg, p=0.03; 9.3(5.4) vs. 10.8(5.7) Wood units, p=0.01, respectively). Renal insufficiency was more common in the SSc patients, but this did not reach statistical significance (8.4% vs. 4.8%, p=0.2). 1- and 2-year outcomes were as follows in the SSc vs. SLE groups: survival 85% and 74% vs. 94% and 84% (p=0.05) (Figure); freedom from hospitalization 69% and 59% vs. 77% and 56% (p=0.7); and freedom from parenteral therapy 91% and 90% vs. 96% and 88% (p=0.6). SLE patients were less likely to die than SSc patients (HR=0.57(0.331.0), p=0.05), but this difference was not statistically significant after adjusting for other variables (HR=0.77(0.431.4), p=0.38).
Figure. Survival in Patients with Systemic Sclerosis- and Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension
Analysis of the REVEAL Registry has demonstrated that poorer survival in patients with SSc compared with SLE-APAH can be explained by differences in clinical parameters, including BNP, DLCO, and renal insufficiency. Further research is necessary to investigate the pathophysiologic role of these markers in SSc-APAH.
To cite this abstract, please use the following information:
Chung, Lorinda, Liu, Juliana, Parsons, Lori, Hassoun, Paul M., McGoon, Michael, Badesch, David, et al; An Analysis of Outcomes in Patients with Systemic Sclerosis- and Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension [abstract]. Arthritis Rheum 2009;60 Suppl 10 :1730