Assesment of the associations of damage index with morbidity and mortality in patient with systemic sclerosis

Purpose: To analyse the association of damage index with morbidity and mortality in patients with Systemic Sclerosis (SSc). Method: The data of 210 SSc patients (followed-up ≥12 months) were analyzed retrospectively. SCTC-DI items and scores were calculated at baseline and last visit. Scores were compared in groups categorized according to limited or diffuse cutaneous SSc (lcSSc (76.2%), dcSSc (23.8%)). The associations of organ damage with morbidity and mortality were evaluated. Results: The most commonly damaged systems were musculoskeletal-skin, GIS, and respiratory. In dcSSc, the frequency of respiratory damage at baseline and follow-up and musculoskeletal-skin damage at follow-up were found to be higher (p=0.003 and p=0.000, p=0.018). Small joint and large joint contractures (musculoskeletal-skin) and BMI/weight loss related damage (GIS) at follow-up, and HRCT with >20% involvement and FVC<70% (respiratory) at baseline and follow-up were more prevalent in dcSSc (p=0.004 and p=0.025, p=0.015, p=0.005 and p=0.000, p=0.013 and p=0.000). All damage scores were worsened during follow-up (renal p=0.026, others p<0.001). Presence of lcSSc was found to be protective against initial respiratory and CVS damages and future high damage, and ACA was found to be against respiratory damage at baseline and at follow-up. In lcSSc, associations of follow-up period >5 years with musculoskeletal-skin; DU and skin thickening in prox MCP (baseline) with peripheral vascular; presence of ILD (baseline) and anti-Scl70 with respiratory; ILD, DU and follow-up period >5 years with GIS; presence of DU and ILD with moderate (6-12 points) and telangiectasia and PAH with high (≥13 points) damage at follow-up were demostrated. In ydSSc, presence of puffy fingers was associated with initial respiratory and follow-up period >5 years with musculoskeletal-skin and respiratory damage. Of the patients, 9 (4.3%) were deceased during follow-up. Increased CVS damage at baseline and follow-up was associated with mortality (p=0.000 and p=0.009). Conclusion: In SSc patients, during 8 years of follow-up, the frequency and severity of organ damage were increased in both cutaneous subtypes, lcSSc and ASA were protective against respiratory damage, and CVS damage was associated with mortality. It was thought that the identification of risky groups in terms of damage could improve survival with early treatment. Key Words: systemic sclerosis, damage score, skin involvement, morbidity, mortality