Chest
Clinical InvestigationsPULMONARY CIRCULATIONHemodynamics and Survival in Patients With Pulmonary Arterial Hypertension Related to Systemic Sclerosis*
Section snippets
Materials and Methods
We performed a retrospective cohort study of consecutive patients with SScPH or PPH who underwent initial pulmonary artery catheterization and vasodilator study from January 1997 to June 2001 at the University of Pennsylvania Pulmonary Vascular Disease Program. The study was approved by the Committee on Studies Involving Human Beings.
The cohort was assembled using the PICARD database, which contains diagnosis data from all inpatient and outpatient medical contacts in the University of
Patient Population
Our cohort was composed of 33 patients with PPH and 22 patients with SScPH. Of patients with PPH, 22 patients (67%) had sporadic pulmonary hypertension, 3 patients (9%) had familial pulmonary hypertension, and 8 patients (24%) had pulmonary hypertension related to anorexigen use. Of patients with SScPH, 16 patients (73%) had SSc with limited cutaneous scleroderma, 4 patients (18%) had SSc with diffuse cutaneous scleroderma, and 2 patients (9%) had SSc in overlap. Patients with missing data were
Discussion
We did not observe significant differences in demographics or hemodynamics between patients with SScPH and patients with PPH. The somewhat lower mean FVC% and TLC% in patients with SScPH are likely due to mild parenchymal lung disease, which may accompany SScPH.20 Of note, pulmonary function testing revealed mild restriction in many of our patients with PPH as well, which has been documented previously.2122 These differences have unclear clinical relevance.
We found a significantly lower Dlco%
Conclusion
Our results suggest that patients with SScPH have a risk of death that is greater than that of patients with PPH after initial evaluation for vasodilator therapy. Although we have not defined the physiologic factor that mediates this increased risk, it is likely that the fibrotic process affects cardiac and/or pulmonary vascular function in patients with SSc.
The worse outcomes in this patient group may have implications in future clinical trials, as this subgroup may not respond to medical
ACKNOWLEDGMENT
The authors thank Dr. Robert Kotloff, Dr. John Hansen-Flaschen, Dr. Yale Enson, and Dr. Robyn Barst for review of this article.
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Supported in part by grants HL07891, and HL04218 (Dr. Taichman) from the National Institutes of Health.