Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


The Pulmonary Arterial Hypertension Quality Enhancement Research Initiative (PAH-QuERI): Comparing Baseline Characteristics, Management and Survival in Patients with Idiopathic PAH (IPAH) to Patients with Systemic Sclerosis Related PAH (SSc-PAH).

Clements2,  Philip J., Casanova1,  Amparo, Mary1,  Tan, Richard4,  Channick, McLaughlin6,  Valerie, Oudiz2,  Ron, Rubin5,  Lew

Canadian Heart Research Center
David Geffen School of Medicine at UCLA
Duke University Medical Center, Durham, NC
Massachusetts General Hospital
University of California at San Diego
University of Michigan Health System

Purpose:

To compare the baseline characteristics, management, and survival in patients with IPAH to patients with SSc-PAH in the prospectively-enrolled PAH-QuERI registry.

Methods:

Between October 2005 and June 2007, physicians at 62 US specialist practices enrolled patients into the registry, entering diagnostic work-up, management and outcome data into an electronic data management system. Automatic queries were generated at each of the prescheduled follow-up visits if the tests recommended by American College of Chest Physicians' guidelines (2007) were not performed at least once.

Results:

287patients with IPAH and 253 with SSc-PAH were entered and followed for one year. At baseline, most of the clinical and laboratory characteristics of the two groups were similar, with only those listed in the table being judged clinically important and statistically different enough to warrant discussion. A few other variables (BMI, heart rate, hemoglobin) were statistically different but the differences were not deemed clinically important.

Both groups were composed largely of middle-aged (57 y/o) Caucasian women (see table). The patients with SSc-PAH were older at the time of PAH diagnosis (56 vs 53 y/o), were more likely to be ANA positive, had lower %DLCO, and more frequently had %FVC/%DLCO ratios > 1.4 and/or DLCO < 55%. The SSc-PAH patients were also more likely to have elevated BNP, shorter 6-minute walk distances (6-MWD), and lower mean pulmonary artery pressures (mPAP) and pulmonary vascular resistance (PVR) than patients with IPAH.

During the one year follow-up period, both groups were managed with prostacyclin derivatives, endothelin-receptor antagonists (ERA) and/or phosphodiesterase-type-5 inhibitors (PDE-5i), singly or in combinations. There were minimal differences in how these drugs were administered over the year of follow-up, with these exceptions: SSc-PAH patients were more likely than IPAH patients to be treated with PDE-5i agents alone or with the combination of ERA and PDE-5i agents.

At 12 months, the mortality rate of the SSc-PAH patients (17%) was significantly greater than that of the IPAH patients (7%, p < 0.001).

Conclusion:

In conclusion: 1) In this prospective registry of IPAH and SSc-PAH patients, the SSc-PAH patients at baseline were more likely to be older and female and to have lower %DLCO and more often had %FVC/%DLCO ratios > 1.4 and DLCO <55%. Although they had better hemodynamics (lower mPAP and lower PVR), they were frequently "sicker" (elevated BNP and shorter 6-MWD). 2) patients with SSc-PAH were more likely to be managed with PDE5i alone or in combination with an ERA than IPAH patients. 3) Survival at one year was significantly lower in the SSc-PAH group than the IPAH group.

Table. Clinically interesting and statistically significant differences in variables at baseline between IPAH and SSc-PAH and in survival at 12 months

Baseline VariablesIPAHSSc-PAHp-value
Age of PAH diagnosis (years old)53560.001
Sex (% females)77%90%<0.001
Abnormal ANA (%)25%85%<0.001
%DLCO6244<0.001
%FVC/%DLCO ratio >1.4 (median values)3467<0.001
DLCO <55% (%)35%70%<0.001
Abnormal BNP (>140 pg/ml) (%)51%64%0.03
6-minute walk distance (6-MWD in meters)3563050.001
Mean pulmonary artery pressure (mPAP in mm Hg)4840<0.001
Pulmonary vascular resistance (PVR in Woods units)8.957.010.02
Survival   
Mortality rate at one year (% death)7%17%<0.001

To cite this abstract, please use the following information:
Clements, Philip J., Casanova, Amparo, Mary, Tan, Richard, Channick, McLaughlin, Valerie, Oudiz, Ron, et al; The Pulmonary Arterial Hypertension Quality Enhancement Research Initiative (PAH-QuERI): Comparing Baseline Characteristics, Management and Survival in Patients with Idiopathic PAH (IPAH) to Patients with Systemic Sclerosis Related PAH (SSc-PAH). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :598
DOI: 10.1002/art.28366

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