Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Systemic sclerosis (SSc) is an immune disorder characterized by vasculopathy and fibrosis. SSc may overlap with another disease such as systemic lupus erythematosus (SLE). Little is known about the epidemiology, clinical characteristics, and survival of SSc-SLE overlap syndrome (also called lupoderma). We evaluated the prevalence of SSc-SLE overlap syndrome, differences in SSc clinical characteristics and survival compared with SSc without SLE.
Methods: A retrospective cohort study was conducted between 1970 – 2017 including subjects who fulfilled the ACR/EULAR classification criteria for SSc and/or who fulfilled the ACR classification criteria for SLE. The primary outcome was the time from diagnosis to death from all causes. Survival was evaluated using Kaplan Meier curves and Cox proportional hazard models. Secondary outcomes included the prevalence of SSc-SLE overlap syndrome, and differences in clinical characteristics.
Results: We identified 1252 subjects (SSc n=1166, SSc-SLE n=86) with a SSc-SLE prevalence of 6.8%. SSc-SLE subjects were more frequently female (92% versus 81%, p=0.02), had lupus anticoagulant (6% versus 0.3%, p<0.001), anticardiolipin antibody (6% versus 0.9%, p<0.001), and pulmonary hypertension (52% versus 31%, p<0.001). SSc-SLE subjects less frequently had calcinosis (13% versus 27%, p=0.007), telangiectasia (49% versus 75%, p<0.001) and diffuse subtype (12% versus 35%, p<0.001). There were no significant differences in the occurrence of renal crisis (7% versus 7%), interstitial lung disease (40% versus 34%), digital ulcers (38% versus 32%), hypertension (19% versus 21%), diabetes (6% versus 6%), dyslipidemia (5% versus 7%) or cancer (7% versus 11%) between groups.
Figure 1. Kaplan Meier curves comparing survival of SSc-SLE overlap to SSc subjects.
The Kaplan Meier curves suggest that SSc-SLE overlap subjects have better survival, but this was not statistically significant (log rank p=0.06). Accounting for female sex and diffuse subtype attenuated survival differences between the two groups (Hazard Ratio 0.70, 95%CI 0.45, 1.11).
Conclusion: SSc-SLE subjects are more frequently female and less frequently have cutaneous manifestations of SSc. However, these subjects should be monitored for serious complications of SSc including pulmonary hypertension, interstitial lung disease, renal crisis and digital ulcers.
To cite this abstract in AMA style:Alharbi S, Ahmad Z, Touma Z, Bookman A, Sánchez-Guerrero J, Johnson S. Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). http://acrabstracts.org/abstract/epidemiology-and-survival-of-systemic-sclerosis-systemic-lupus-erythematosus-overlap-syndrome/. Accessed November 22, 2017.
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ACR Meeting Abstracts - http://acrabstracts.org/abstract/epidemiology-and-survival-of-systemic-sclerosis-systemic-lupus-erythematosus-overlap-syndrome/