Phenotypes of patients with systemic sclerosis in the Chinese Han population: a cluster analysis

Abstract

ObjectiveSystemic sclerosis (SSc) is a heterogeneous connective tissue disease that is commonly subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) based on the extent of skin involvement. This subclassi cation may not re ect the full range of clinical phenotypic variation. This study aimed to investigate clinical features and aggregation of patients with SSc in China based on SSc manifestations and organ involvements. MethodsIn total 287 SSc patients were included in this cross-sectional study. A cluster analysis was applied according to 13 clinical and serologic variables to determine subgroups of patients. Survival rates between obtained clusters and risk factors affecting prognosis were also compared. ResultIn this study, six clusters were observed: cluster 1 (n = 66) represented the skin type, with all patients showing skin sclerosis. In cluster 2 (n = 56), most patients had vascular and articular involvement. Cluster 3 (n = 14) individuals mostly had cardiac and pulmonary involvement. In cluster 4 (n = 52), the gastrointestinal type, 50 patients presented with gastritis. In cluster 5 (n = 50), patients barely had any major organ involvement. Cluster 6 (n = 49) included six patients with renal insu ciency, 28 patients with muscle pain and 42 patients with Raynaud's symptoms. ConclusionThe results of our cluster analysis study implied that limiting SSc patient subgroups to those based only on skin involvement might not capture the full heterogeneity of the disease. Organ damage and antibody pro les should be considered when identifying homogeneous patient groups with a speci c prognosis.