Abstract
BackgroundSystemic sclerosis (SSc) is a severe and highly heterogeneous disease. The modified Rodnan skin score (mRSS) is a widely used tool for the assessment of the extent and degree of skin thickness. This study aimed to identify the classes of patients with early similar skin thickening trajectories without any a priori assumptions and study their associations with organ involvement and survival.MethodsFrom the French SSc national cohort, patients with a disease duration of less than 2 years at inclusion and with at least 2 mRSS available within the first 4 years of follow-up were enrolled. Classes of patients with similar mRSS trajectories were identified based on a latent class mixed model. The clinical characteristics and survival rate were compared between the obtained classes.ResultsA total of 198 patients fulfilled the inclusion criteria, with a total of 641 mRSS available. The median disease duration and follow-up were 0.8 (interquartile range 0.4; 1.2) and 6.3 (3.8; 8.9) years, respectively. Individual trajectories of mRSS were highly heterogeneous between patients. Models with 1–6 latent classes of trajectories were sequentially assessed, and the 5-class model represented the best fit to data. Each class was characterized by a unique global trajectory of mRSS. The median disease duration did not differ significantly between classes. Baseline organ involvement was more frequent in classes with significant change over time (classes 2–5) than in class 1 (low baseline mRSS without significant change over time). Using Cox regression, we observed a progressively increasing risk of death from classes 1 to 5.ConclusionsEarly identification of clinical phenotype based on skin thickening trajectories could predict morbi-mortality in SSc. This study suggested that mRSS trajectories characterization might be pivotal for clinical practice and future trial designs.
Highlights
Systemic sclerosis (SSc) is a severe and highly heterogeneous disease
Deciphering the skin thickening heterogeneity is of utmost importance considering the wide use of modified Rodnan skin score (mRSS) as the primary outcome and the three recent negative clinical trials to prove a benefice on skin thickening in SSc [11,12,13]
Data were extracted in August 2015, and patients were eligible for the present analysis if (i) the ACR 1980 preliminary classification criteria [14] and/or 2013-ACR/EULAR SSc classification criteria [15] were fulfilled, (ii) inclusion visit occurred less than 2 years after the first onset of non-Raynaud phenomenon (RP) symptom, and (iii) the baseline mRSS and at least 1 mRSS during follow-up were available
Summary
Systemic sclerosis (SSc) is a severe and highly heterogeneous disease. The modified Rodnan skin score (mRSS) is a widely used tool for the assessment of the extent and degree of skin thickness. This study aimed to identify the classes of patients with early similar skin thickening trajectories without any a priori assumptions and study their associations with organ involvement and survival. The two subgroups of lcSSc patients were as follows: one where the skin clinical phenotype subsequently became diffuse, and the remaining one was limited throughout the follow-up. These studies underlined the well-known heterogeneity of the skin thickening evolution in SSc, and the complexity of the relations between mRSS at baseline and the skin thickening course (improvers/non-improvers and STPR). We aimed to identify the early mRSS longitudinal trajectories in SSc patients from the prospective French SSc national database without any a priori assumptions and to examine their associations with organ involvement and survival
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