Skin outcomes in limited systemic sclerosis: data of tertiary center

Abstract

Introduction: Systemic sclerosis (SSc) is a heterogeneous autoimmune disorder of unknown aetiology, which may involve the skin and internal organs to varying degrees and with different courses. The extent of skin involvement has a major impact on disease prognosis in the diffuse cutaneous subtype of SSc. However, there is scarce data regarding skin course in the limited cutaneous form (lcSSc). Our aims were to investigate skin involvement courses, and identify predictors for these courses, in lcSS patients.Methods: We performed a retrospective analysis including patients with lcSSc fulfilling the 2103 ACR/EULAR Classification Criteria for Systemic Sclerosis. Skin improvement, progression or stable course was defined by a cut-off of 3.5 points change in mRSS from baseline to last appointment. The European Scleroderma Trial and Research group Disease Activity Index (EUSTAR-DAI), clinical and laboratorial features were assessed. Baseline characteristics were compared between groups using chi-square and independent samples t-test, as appropriate. Logistic regression analysis was performed to identify possible predictive factors associated with skin course involvement.Results: We included 71 lcSSc patients [87.2% female, mean age at diagnosis 55.7 (SD 11.6) years]. The mean follow-up time was 15.5±9.2 years from baseline to last appointment. Maintenance of mRSS was observed in 65.2% (n=45), improvement in 27.6% (n=21) and progression in 7.2% (n=5) of the patients. A high baseline mRSS was associated with an improvement course (p<0.001). LcSSc patients with an inactive disease (EUSTAR-DAI<2.5) at baseline tend to have a stable mRSS course (p=0.001). In multivariate analysis, a high baseline skin score was predictor of skin improvement course during the follow-up (OR [95 IC]: 21.44 [5.86-78.49], p<0.001).Conclusions: LcSSc patients with advanced skin fibrosis at baseline are more likely to improve skin score than patients with milder skin fibrosis. Recognizing distinct patterns of mRSS course could help tailoring clinical management and cohort enrichment. Studies with a larger sample size will be needed to shed some light on skin involvement and its prognostic value in lcSSc.

Introduction: Systemic sclerosis (SSc) is a heterogeneous autoimmune disorder of unknown aetiology, which may involve the skin and internal organs to varying degrees and with different courses. The extent of skin involvement has a major impact on disease prognosis in the diffuse cutaneous subtype of SSc. However, there is scarce data regarding skin course in the limited cutaneous form (lcSSc). Our aims were to investigate skin involvement courses, and identify predictors for these courses, in lcSS patients.Methods: We performed a retrospective analysis including patients with lcSSc fulfilling the 2103 ACR/EULAR Classification Criteria for Systemic Sclerosis. Skin improvement, progression or stable course was defined by a cut-off of 3.5 points change in mRSS from baseline to last appointment. The European Scleroderma Trial and Research group Disease Activity Index (EUSTAR-DAI), clinical and laboratorial features were assessed. Baseline characteristics were compared between groups using chi-square and independent samples t-test, as appropriate. Logistic regression analysis was performed to identify possible predictive factors associated with skin course involvement.Results: We included 71 lcSSc patients [87.2% female, mean age at diagnosis 55.7 (SD 11.6) years]. The mean follow-up time was 15.5±9.2 years from baseline to last appointment. Maintenance of mRSS was observed in 65.2% (n=45), improvement in 27.6% (n=21) and progression in 7.2% (n=5) of the patients. A high baseline mRSS was associated with an improvement course (p<0.001). LcSSc patients with an inactive disease (EUSTAR-DAI<2.5) at baseline tend to have a stable mRSS course (p=0.001). In multivariate analysis, a high baseline skin score was predictor of skin improvement course during the follow-up (OR [95 IC]: 21.44 [5.86-78.49], p<0.001).Conclusions: LcSSc patients with advanced skin fibrosis at baseline are more likely to improve skin score than patients with milder skin fibrosis. Recognizing distinct patterns of mRSS course could help tailoring clinical management and cohort enrichment. Studies with a larger sample size will be needed to shed some light on skin involvement and its prognostic value in lcSSc.