Background In systemic sclerosis (SSc), studies showed that permenant organ damage may exist very early in the disease course.

Objectives To reveal the differences between diffuse cutaneous and limited cutaneous SSc (dcSSc and lcSSc) patients regarding the progress of disease related organ damage over time.

Methods Medical records of consecutive 210 patients(91.9% women) who fulfilled SSc classification criteria and had a follow-up period of at least 12 months were retrospectively evaluated. ‘Scleroderma Clinical Trials Consortium Damage Index‘ score* was calculated initially and after a follow-up period of 101±70 months in dcSSc and lcSSc patients.

Results In dcSSc; joint contractures, low BMI/weight loss, and dependence on oxygen, ILD with>20% extent on HRCT and FVC<70% were more frequent at follow-up, total damage scores were higher at baseline and follow-up (3.1±2.8 vs 1.9±2.5,p=0.004 and 9.7±5.9 vs 5.8±4.6,p<0.000) (Table 1, Figure1). In dcSSc; presence of puffy fingers was associated with initial respiratory damage (OR:3.88,p=0.008) and ILD with musculoskeletal-skin damage at follow-up (OR=3.547, p=0.02). In lcSSc; follow-up period of >5 years was associated with musculoskeletal-skin damage (OR=10.17,p=0.002), presence of skin thickening proximal to the MCP with peripheral vascular damage (OR=8.48,p=0.000 and OR=2.87,p=0.047), presence of anti-Scl70 with respiratory damage (OR=4.84, p=0.008), presence of ILD, DU and follow-up period of >5 years with GIS damage(OR=2.48,p=0.001, OR=1.79,p=0.029 and OR=5.77,p=0.000) at follow-up. In lcSSc, the presence of DU and ILD were associated factors with moderate damage (6-12 points)(OR=2.41,p=0.000 and OR=1.94,p=0.006), telangiectasias and PAH with high damage (≥13 points) (OR=2.42,p=0.041 and OR=2.87,p=0.004) at follow-up. Of the patients 4.3%(n=9) died during follow-up; cardiovascular damage (baseline and follow-up) was associated with mortality (5.5% vs.55.6%, p<0.000, and 15.4% vs.55.6%, p=0.009).

Conclusion In SSc patients, the frequency and severity of organ damage were increased in both cutaneous subtypes during 8 years of follow-up. Presence of ILD, DU, telangiectasia and PAH and long duration of follow-up period were prominent factors associated with damage. Cardiovascular damage was found to be associated with mortality. The identification of risk groups in different cutaneous subtypes of SSc and early efficacious treatment strategies could improve survival.

Reference [1]Ferdowsi N, et al. Ann Rheum Dis. 2019;78:807-816.

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Figure 1

: Progress of total damage scores in SSc patients with different cutaneous subtypes

Acknowledgements: NIL.

Disclosure of Interests None Declared.