RT Journal Article
SR Electronic
T1 Systemic sclerosis associated with pulmonary arterial hypertension is most severe in African American patients
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p2336
VO 38
IS Suppl 55
A1 Hala El Chami
A1 Aranzazu Campo
A1 Stephen Mathai
A1 Ari Zaiman
A1 Danielle Boyce
A1 Noah Lechtzin
A1 Laura Hummers
A1 Reda Girgis
A1 Paul Hassoun
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p2336.abstract
AB Background: African Americans (AA) with Systemic Sclerosis (SSc) have a lower age at disease onset, a higher frequency of diffuse (vs. limited) skin involvement, and an overall worse prognosis compared to other ethnic groups.Objective: To estimate the differences in hemodynamics, clinical presentation and survival between AA and Caucasians with SSc associated pulmonary arterial hypertension (SSc-PAH).Methods: We conducted a study of 75 consecutive SSc patients diagnosed with PAH by heart catheterization (mPAP≥25mmHg and PCWP≤ 15mmHg) at a single center. Kaplan Meier estimates were calculated and mortality risk factors were analyzed.Results: The cohort included 9 AA and 66 Caucasians. Forty one (54.7%) patients were in WHO functional class III or IV. There was no difference in age between the 2 groups (59±13 vs. 61±11 years P=0.7), or disease duration (11±11 vs. 12.7±13 years, P=0.7). AA patients had a higher prevalence of systemic hypertension (66.7% vs. 30.3%; P=0.03), higher mPAP (51±12 vs. 41.8±11 mmHg; P=0.02) and PVR (14.8±9.5 vs. 8.04±4.6 Wood units; P=0.01), and lower SVI (24±10.5 vs. 32±10 mL/m2, P=0.054), but similar PCWP (11.6±2.6 vs. 10.18±3.4 mmHg, P=0.25). Renal dysfunction (defined as eGFR <60) (33.3% vs. 45.5%; P=0.49), extent of disease (88.9% had limited disease vs. 89.2%; P=0.9) and treatment between AA patients and Caucasians were not different. Median survival did not differ between the two groups (3.2 vs. 4.1 years, P=0.4).Conclusions: SSc-PAH is significantly more severe in AA compared to Caucasians and is characterized by right ventricular dysfunction.