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Overlap syndromes in systemic sclerosis
Ewa Wielosz1, Maria Majdan1, Magdalena Dryglewska1
1Department of Rheumatology and Connective Tissue Diseases, Medical University of Lublin, Lublin, Poland.
Postepy Dermatologii I Alergologii
|July 17, 2018
Summary
Overlap syndromes (OS) are more common in limited cutaneous systemic sclerosis (lcSSc) than diffuse cutaneous systemic sclerosis (dcSSc). Manifestations and outcomes differ significantly between systemic sclerosis (SSc) and OS patients.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Systemic sclerosis (SSc) disease course varies between overlap syndromes (OS) and SSc groups.
- Understanding these differences is crucial for patient management.
Purpose of the Study:
- To compare the prevalence of OS in limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc).
- To analyze clinical and serological manifestations in SSc versus OS patients.
Main Methods:
- 126 European Caucasian SSc patients (dcSSc n=57, lcSSc n=69) were analyzed.
- Patients met American College of Rheumatology (ACR) criteria for SSc.
- Groups were defined by SSc subtype, coexisting connective tissue diseases (CTDs), and clinical/serological features.
Main Results:
- 22% of SSc patients had more than one CTD (OS).
- OS prevalence was higher in lcSSc (33%) vs. dcSSc (8%).
- Mortality and digital ulcers were higher in SSc; kidney involvement and arthritis were lower compared to OS. Anti-topoisomerase I (a-Scl-70) was higher in SSc, while anti-PM/Scl and anti-Ro-52 were lower compared to OS.
Conclusions:
- Overlap syndromes are more frequent in lcSSc than dcSSc.
- Distinct clinical courses and internal organ involvement patterns exist between SSc and OS.
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