PT  - JOURNAL ARTICLE
AU  - Hala El Chami
AU  - Aranzazu Campo
AU  - Stephen Mathai
AU  - Ari Zaiman
AU  - Danielle Boyce
AU  - Noah Lechtzin
AU  - Laura Hummers
AU  - Reda Girgis
AU  - Paul Hassoun
TI  - Systemic sclerosis associated with pulmonary arterial hypertension is most severe in African American patients
DP  - 2011 Sep 01
TA  - European Respiratory Journal
PG  - p2336
VI  - 38
IP  - Suppl 55
4099  - http://erj.ersjournals.com/content/38/Suppl_55/p2336.short
4100  - http://erj.ersjournals.com/content/38/Suppl_55/p2336.full
SO  - Eur Respir J2011 Sep 01; 38
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.