Background/Purpose: To estimate whether severity of skin thickness is associated with disease severity, function, quality of life (QoL) and progression of internal organ involvement over 12 months in early diffuse cutaneous systemic sclerosis (dcSSc).

Methods: We included dcSSc patients from the Canadian Scleroderma Research Group (CSRG) database with disease duration of ≤5 years from the onset of first non-Raynaud’s symptom at initial visit and 1-year follow-up (FU) . We assessed severity of skin involvement with the modified Rodnan skin score (mRSS); internal organ involvement with the Medsger organ severity scores; global measures of disease status with patient- and physician-reported 11 point numerical rating scales (NRS); disease activity with the European Scleroderma Study Group (EScSG) activity index; function with the HAQ and S-HAQ 11 point NRS scales; and QoL with the SF-36. Bivariate, ANOVA, linear and logistic regression analyses were used to study the associations between mRSS and outcomes, adjusted for potential confounding factors.

Results: At baseline (N=204, 74% female, mean age 51±12 yrs, disease duration 2.1±1.3 yrs), higher mRSS was significantly associated with worse total disease severity (Medsger’s score without skin component, physician and patient global NRS, S-HAQ overall disease rating), functional disability (HAQ-DI) and worse QoL (SF-36 PCS) (p<0.0001 for all). Higher mRSS was also associated with the presence and severity of tendon involvement (p<0.0001), severity of gastrointestinal disease (p=0.021), S-HAQ digital ulcer severity (p=0.008) and S-HAQ intestinal problems (p=0.008) by adjusted regression analysis.

At 1-year FU visit (N=174, 74% female, mean age 51±12 yrs, disease duration 2.1±1.3 yrs) we found a significant reduction in mRSS (22.51±9.27 vs 19.42±10.45, p=0.006). There was also a decrease in disease activity by both EScSG-activity index (3.06±1.8 vs 2.41±1.8, p=0.003) and physician-assessed disease activity on NRS scale (4.61±2.37 vs 3.52±2.17, p<0.0001). There were no changes in other measures of disease characteristics.

We also examined the associations between baseline mRSS and outcomes at 1-year FU visit using adjusted regression analysis. Higher baseline mRSS was associated with worse HAQ-DI (p=0.0001) and SF-36 PCS (p=0.0001), higher total Medsger’s severity score (p=0.014), physician-assessed disease damage (p=0.001), severity (p=0.008) and joint involvement (p=0.0001) at FU. Baseline mRSS was not associated with presence/severity of kidney, lung or heart disease at baseline or 1-year FU visit, nor predictive of new/progression of organ involvement over time.

Conclusion: Greater skin involvement at baseline was associated with worse joint and GI involvement, worse function and QoL. Baseline mRSS also predicted function, severity of joint involvement and QoL at 1-year FU. No association between skin involvement and burden of other organ-based complications was found, but follow-up was limited to 1-year in this analysis.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-association-between-the-extent-of-skin-thickness-and-organ-involvement-function-and-quality-of-life-in-early-diffuse-cutaneous-systemic-sclerosis/