Investigators have revealed that although less common, systemic sclerosis sine scleroderma can still cause severe disease, according to an article published in Medscape. In a new study published by Lescoat et al in JAMA Dermatology, investigators used the EUSTAR database to identify over 4,200 patients with systemic sclerosis. They then compared the skin manifestations and survival times of patients who had systemic sclerosis (n = 376) sine scleroderma with patients who had two other subtypes—diffuse cutaneous systemic sclerosis (n = 708) and limited cutaneous systemic sclerosis (n = 708). The investigators found that nearly 50% of patients with systemic sclerosis sine scleroderma had interstitial lung disease and those with internal dysfunction—specifically diastolic heart dysfunction—had associated cutaneous findings. In addition, the investigators discovered that patients in the sine scleroderma group had a lower prevalence of digital ulcers and pitting scars than the other groups. Further, the analysis also showed that 63.8% of patients in the sine scleroderma group displayed puffy fingers vs 82.4% of patients in the limited cutaneous group and 87.6% of those in the diffuse cutaneous group. After a follow-up of 15 years, the investigators found that patients with sine scleroderma had survival rates of 92.4% vs 69.4% and 55.5% among those with limited cutaneous and diffuse cutaneous disease, respectively. The investigators concluded that although the survival rates were higher in those with systemic sclerosis sine scleroderma, these patients also presented with higher rates of internal manifestations. They emphasized the need to better detect and diagnose systemic sclerosis and provide timely treatments to improve patient outcomes.
July 26, 2023