Background/Purpose: Pulmonary hypertension (PH) is one of the leading causes of mortality in patients with systemic sclerosis (SSc). Active screening detects PH earlier and may improve survival in SSc patients. Our objective was to evaluate the development of PH in high risk SSc patients over time who initially had a negative right heart catheterization (RHC).

Methods: Subjects with a diagnosis of SSc based on the 2013 ACR/EULAR criteria who did not have a prior diagnosis of PH who underwent a resting RHC due to increased risk based on 2013 recommendations for screening and detection of connective tissue disease-PAH¹, which includes DETECT, were included in this prospective observational cohort. Those subjects who did not have a diagnosis of PH according to resting baseline RHC with mean pulmonary artery pressure (mPAP) < 25mmHg underwent subsequent screening resting RHC approximately 24 months later, or earlier if they developed new symptoms and/or change in non-invasive testing parameters.

Results: Sixty-seven subjects with SSc who underwent baseline resting RHC were included. Ninety-percent of subjects were female, 82% were Caucasian, 12% were African American, 66% had limited cutaneous SSc, and 34% had diffuse cutaneous SSc. Mean (SD) age was 61.4 (9.6) years, SSc disease duration was 13.2 (11.4) years, FVC% predicted was 79.9 (20.3), DLCO% predicted was 57.7 (18.4), and FVC/DLCO was 1.5 (0.6). At baseline RHC, 24% had normal mPAP (≤ 20 mmHg), 34% had borderline mPAP (BoPAP, 21-24 mmHg), and 42% had PH (≥ 25mmHg) (Figure 1). No patients with normal mPAP or BoPAP were treated with PAH specific therapies.  Eleven subjects, 2 with normal mPAP and 9 with BoPAP, underwent repeat RHC; 3 subjects with BoPAP progressed to PH, 3 subjects with BoPAP decreased to normal mPAP, 3 subjects remained BoPAP, 1 subject with normal mPAP progressed to BoPAP and 1 subject with normal mPAP remained at normal mPAP (Figure 2).

Conclusion: Use of 2013 recommendations resulted in identification of a high proportion of subjects with normal mPAP or BoPAP. Approximately 1/3 of patients with BoPAP progress to PH after 2 years, which is different than previously reported^2,3. This may be due to universal PAH screening of SSc patients at our center on annual basis.

References:

1. Khanna et al. Arthritis Rheum. 2013.

2. Bae et al. Ann Rheum Dis. 2012.

3. Valerio et al. Arthritis Rheum. 2013.