Late-age onset systemic sclerosis-clinical and serological characteristics

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis and vascular abnormalities.
• The clinical presentation and outcomes of SSc can vary significantly based on age of onset.
• Understanding differences between early-age onset SSc (EAO SSc) and late-age onset SSc (LAO SSc) is crucial for tailored management.

Purpose of the Study:

• To compare the clinical course and serological profiles of LAO SSc (onset > 60 years) with EAO SSc (onset < 60 years).
• To identify specific organ involvement, serological markers, and comorbidities associated with LAO SSc.

Main Methods:

• Retrospective analysis of 157 SSc patients meeting ACR/EULAR classification criteria.
• Categorization into LAO SSc (n=39) and EAO SSc (n=118) groups based on age at diagnosis.
• Comparison of SSc subtype, internal organ involvement, malignancy, mortality, and serological profiles between groups.

Main Results:

• LAO SSc showed significantly higher prevalence of pulmonary arterial hypertension (PAH), heart involvement, and renal involvement compared to EAO SSc.
• Arthralgias were less common in LAO SSc (p=0.02).
• LAO SSc exhibited lower prevalence of anti-RNA polymerase III (p=0.008) and anti-PM/Scl antibodies (p=0.048), but higher anti-Th/To antibodies (p=0.014) than EAO SSc.

Conclusions:

• Significant clinical and serological differences exist between LAO SSc and EAO SSc.
• Internal organ morbidity (PAH, renal, cardiac) is more common in LAO SSc, suggesting a role for age-related comorbidities.
• Distinct antibody profiles in LAO SSc may reflect different pathogenic mechanisms or disease manifestations.