Clinical trajectories and predictors of hand function deterioration in systemic sclerosis

Abstract Background/Aims Hand impairment is a major driver of disability and reduced quality of life in Systemic Sclerosis (SSc) patients. It is multifactorial and the risk and predictability of functional deterioration are unknown. The aim of this study is to describe hand function changes and predictors of functional deterioration in an observational cohort of SSc patients. Methods Consecutive SSc patients were longitudinally evaluated every 6 months for 3 years for SSc-related hand involvement. Patient-reported hand symptoms were captured with Cochin Hand Disability Score (CHFS), Raynaud Condition Score (RCS) and overall pain intensity on a visual analogue scale (VAS pain). Their minimal clinical important difference (MCID) and patient-acceptable symptom state (PASS) were evaluated according to validated thresholds. Clinical association with CHFS change over time and 36-month clinically meaningful worsening (MCID-Wor) were assessed via ANOVA and binomial logistic regression, respectively. Results One-hundred-seventy-six SSc patients were enrolled in this analysis, 13.6% of them were male and the mean age was 55.1± 12.3 years, the median disease duration was 5.0 years and 31.8% presented a diffuse cutaneous variant. Overall, CHFS worsened over time (p < 0.001); 34.1% of patients reported a clinically significant worsening by the end of follow-up. Accordingly, the proportion of participants reporting CHFS≥PASS increased from 26.1% at baseline to 34.7% at the last follow-up. MCID-W after 36 months was associated with diffuse cutaneous variant [OR 2.44 (95% CI 1.27-4.76), p = 0.008], baseline active-late capillaroscopy pattern [OR 3.00 (95% CI 1.17-7.70), p = 0.013], VAS pain [OR 1.02 (95% CI 1.01-1.03), p = 0.003], RCS [OR 1.03 (95% CI 1.02-1.04), p < 0.001], forearm-hand-finger skin score [OR 1.12 (95% CI 1.00-1.26), p = 0.044], history of digital ulcers [OR 2.39 (95% CI 1.24-4.61), p = 0.008] or flexor contractures [OR 2.52 (95% CI 1.29-4.92), p = 0.007], as well as baseline CHFS [OR 1.03 (95% CI 1.01-1.05), p = 0.001]. A regression model based on these clinical variables could correctly classify 76.7% of cases. Conclusion SSc-related hand disability affects more than one-third of patients and tends to worsen over time in both cutaneous subsets despite the current standard of care. Identifying patients at risk of deteriorating hand function should inform targeted intervention strategies to prevent hand disability in SSc. Disclosure E. De Lorenzis: None. V. Kakkar: None. S. Di Donato: None. M. Wilson: None. L. Green: None. B. Alcacer-Pitarch: None. F. Del Galdo: Other; FDG received consultancies and research support from Abbvie, AstraZeneca, Boheringer-Ingelheim, Capella, Chemomab, Ergomed, Janssen, Kymab, Mitsubishi-Tanabe.