PURPOSE: Most studies suggest that ethnic factors impact significantly on prognosis and survival in Systemic Sclerosis (SSc). African Americans (AA) 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.
METHODS: We conducted a study of 95 consecutive SSc patients newly diagnosed with pulmonary arterial hypertension (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 14 AA and 81 Caucasians. Fifty-two (55.9%) patients were in WHO functional class (FC) III or IV. AA tended to be younger (56 ± 12 years vs. 60 ± 12 years, P=0.08), but there was no differential in WHO FC between the two groups. AA patients had a higher prevalence of systemic hypertension (HTN) (64.3% vs. 28.2%; P=0.01). However, there were no differences in hemodynamics (mPAP 40 ± 10 vs. 40 ± 11; P=0.71, CI 2.41 ± 0.80 vs. 2.61 ± 0.74; P= 0.28, PVR 8.6 ± 6.3 vs. 7.5 ± 4.5; P=0.76, respectively), age of SSc diagnosis (43.1 ± 18.3 years vs. 50.8 ± 13.7 years; P=0.1), extent of disease (84.6% had limited disease vs. 87.5%; P=0.67) and renal dysfunction (defined as eGFR < 60) (28.6% vs. 43.2%; P=.38) between AA patients and other ethnic groups. Kaplan-Meier survival estimates were 82%, 71%, 66%, 53%, and 39% at 1, 2, 3, 4 and 5 years respectively, with no significant difference between the 2 groups (p=0.62).
CONCLUSION: Although AA tended to present with PAH at a younger age, there were no differences in PAH severity and hemodynamics between AA and other ethnic groups.
CLINICAL IMPLICATIONS: SSc-associated PAH is not more severe in AA compared to Caucasians, a finding that needs to be confirmed in larger cohorts.
DISCLOSURE: Hala El Chami, No Financial Disclosure Information; No Product/Research Disclosure Information