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.

Stanford University, Palo Alto, CA,
ICON Clinical Research, San Francisco, CA,
Johns Hopkins University, Baltimore, MD,
Mayo Clinic, Rochester, MN,
University of Colorado, Denver, CO

Purpose:

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.

Methods:

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).

Results:

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(0.33–1.0), p=0.05), but this difference was not statistically significant after adjusting for other variables (HR=0.77(0.43–1.4), p=0.38).

Figure. Survival in Patients with Systemic Sclerosis- and Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension

Conclusion:

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
DOI: 10.1002/art.26804

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