Clinical trajectories of multifactorial hand function deterioration in systemic sclerosis.

Abstract

Hand function deterioration is a major multifactorial driver of disability in Systemic Sclerosis (SSc) whose rate has been poorly described. The aim of this study is to describe incidence and risk factors of hand functional worsening in a longitudinal, multicenter, observational SSc cohort. Hand involvement and disability were evaluated in consecutively enrolled patients for 24 months. Patient-reported hand impairment was captured with Cochin Hand Disability Score (CHFS) and the corresponding minimal clinical important differences (MCID) and patient acceptable symptom state (PASS). Clinical association with CHFS change over time and clinically meaningful worsening (MCID-Worsening) were investigated. Three-hundred-ninety-six patients from 10 centres were evaluated and 201 SSc (age 55.7�12.2 years, male 13.4%) were included in the final analysis. Median (IQR) disease duration was 5 (2-11) years while the proportion of patients with diffuse cutaneous variant was the 29.9%. Fifty-six (27.8%) patients had a CHFS ?PASS at baseline. CHFS increased over time 35.8% of patients reaching CHFS?PASS (p<0.001), and 52.2% of patients reporting MCID-Worsening at 24 months. A LASSO model simultaneously exploring the effects of multiple baseline clinical variables showed that MCID-Worsening was associated with male gender, LeRoy diffuse variant, late capillaroscopy pattern, shorter disease duration, absence of digital ulcers, presence of tenosynovitis, pain, Raynaud�s phenomenon, and global and hand disability severity, together with treatment with immunosuppressants, vasoactive medications, and second-line analgesics. Hand function tends to deteriorate over time in one SSc patient in two despite available therapies and clinical assessment support risk stratification. These results pave the way to inform design of intervention studies aimed at improving the outcome of this major driver of disability in SSc.