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Abstract Number: 2982

Morbidity and Mortality of Scleroderma in African Americans

Duncan F. Moore1, Elisabeth Kramer1, Rami Eltaraboulsi2 and Virginia D. Steen1, 1Division of Rheumatology, Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, 2MedStar Georgetown University Hospital, Washington, DC

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: African-Americans, morbidity and mortality, scleroderma and systemic sclerosis

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Session Information

Date: Wednesday, November 8, 2017

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics III

Session Type: ACR Concurrent Abstract Session

Session Time: 11:00AM-12:30PM

Background/Purpose:
Retrospective cohorts have demonstrated that African Americans (AAs) with scleroderma are more likely to have severe disease and higher mortality than non-AAs. A prior study concluded that this excess risk was not fully explained by differences in rates of diffuse disease and autoantibody status (Gelber 2013). Our study sought to further evaluate this risk among matched AAs and non-AAs at our site.

Methods:
A single-center, retrospective study comparing AA and non-AA patients with scleroderma was performed. Patients were evaluated by the senior author between 2008 and 2016. AA and non-AAs were matched based on sex, age at first visit, date of first visit, disease duration at first visit, and limited vs. diffuse cutaneous disease. Demographic, serologic, and clinical features were compared. Mortality risks were assessed in an unadjusted manner and by a Cox proportional hazards model with covariates of race, median household income by ZIP code, marital status, education level, employment status, and insurance type.

Results:
AAs comprised 203 of the 402 patients analyzed. The groups were statistically similar regarding all factors by which they were matched. Table 1 shows our findings. AAs had significantly reduced FVC and DLCO, more severe fibrosis per CT, a higher prevalence of any type of pulmonary hypertension, and more severe cardiac involvement. As expected, autoantibody profile statistically differed among the two groups (p<0.001). Death during follow-up was 21.2% in AAs vs. 11.1% in non-AAs (p=0.006). AA status demonstrated an unadjusted hazard ratio for death during follow-up of 2.037 (p=0.007), but when adjusted for the aforementioned socioeconomic covariates, the hazard ratio of AA status declined to 1.256 (95% CI 0.494 – 3.191, p=0.633). The only statistically significant covariate was median income in thousands of dollars by ZIP code, with a hazard ratio of 0.983 (95% CI 0.968 – 0.999, p=0.033).

Conclusion:
African American patients with scleroderma have more severe pulmonary disease and a higher unadjusted risk of mortality than matched non-African Americans. Adjusting for available socioeconomic factors, African American race was not a significant risk factor for mortality, but lower median household income by ZIP code was an independent risk factor for increased mortality.

Table 1 – Morbidity and mortality in African Americans and non-African Americans
African American (n = 203) Non-African American (n = 199) p-value
Sex (female) 176 (86.7) 173 (86.9) 0.944
Age at first visit, mean ± SD 47.4 ± 13.3 48.5 ± 13.0 0.379
Disease duration at first visit
(years), mean ± SD
7.7 ± 8.1 8.3 ± 9.6 0.512
SSc type (diffuse) 97 (47.8) 81 (40.7) 0.121
Autoantibody < 0.001
anti-centromere 15 (7.4) 43 (21.6) < 0.001
anti-Scl70 43 (21.2) 41 (20.6) 0.886
anti-U1RNP 26 (12.8) 10 (5.0) 0.006
isolated nucleolar ANA 50 (24.6) 32 (16.1) 0.033
anti-RNA polymerase III 7 (3.4) 29 (14.6) < 0.001
non-specific SSc ANA 46 (22.7) 27 (13.6) 0.018
negative ANA 11 (5.4) 9 (4.5) 0.680
no result 5 (2.5) 8 (4.0) 0.378
Inflammatory arthritis 33 (16.3) 43 (21.6) 0.171
Tendon rubs 25 (12.3) 40 (20.1) 0.034
Telangiectasia 39 (19.2) 101 (50.8) < 0.001
Calcinosis 10 (4.9) 33 (16.6) < 0.001
Skin score, mean ± SD 14.1 ± 13.2 14.4 ± 12.6 0.803
FVC (% predicted), mean ± SD 68.4 ± 20.4 84.1 ± 48.7 < 0.001
DLCO (% predicted), mean ± SD 45.8 ± 19.8 63.7 ± 20.9 < 0.001
Fibrosis per CT 0.002
no fibrosis 38 (18.7) 60 (30.2) 0.008
mild/moderate fibrosis 77 (37.9) 67 (33.7) 0.373
severe fibrosis 22 (10.8) 6 (3.0) 0.002
not performed 66 (32.5) 66 (33.2) 0.889
Pulmonary hypertension by
right heart catheterization
42 (20.7) 25 (12.6) 0.029
Severe cardiac involvement 35 (17.2) 17 (8.5) 0.009
Renal crisis 17 (8.4) 6 (3.0) 0.021
Death during follow-up 43 (21.2) 22 (11.1) 0.006
Duration from diagnosis to death or
end of study (years), mean ± SD
9.5 ± 7.3 9.5 ± 8.9 0.920

Disclosure: D. F. Moore, None; E. Kramer, None; R. Eltaraboulsi, None; V. D. Steen, None.

To cite this abstract in AMA style:

Moore DF, Kramer E, Eltaraboulsi R, Steen VD. Morbidity and Mortality of Scleroderma in African Americans [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/morbidity-and-mortality-of-scleroderma-in-african-americans/. Accessed .
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