Open Access Rheumatology: Research and Reviews (Sep 2022)
Predicting the Progression of Very Early Systemic Sclerosis: Current Insights
Abstract
Chiara Bellocchi,1,2 Augustine Chung,3 Elizabeth R Volkmann4 1Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, University of Milan, Milan, Italy; 2Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy; 3Division of Pulmonary and Critical Care, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA; 4Division of Rheumatology, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USACorrespondence: Elizabeth R Volkmann, Division of Rheumatology, Department of Medicine, University of California, Los Angeles, David Geffen School of Medicine, 1000 Veteran Avenue, Ste 32-59, Los Angeles, CA, 90095, USA, Tel +1 310-825-5800, Fax +1 310-206-5088, Email [email protected]: Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease with distinct pathological hallmarks (ie, inflammation, vasculopathy, fibrosis) that may predominate at different stages in the disease course with varying severity. Initial efforts to classify patients with SSc identified a subset of patients with very early SSc. These patients possessed signs of SSc (eg, Raynaud phenomenon, SSc specific autoantibodies and/or nailfold capillary abnormalities) without fulfilling complete SSc classification criteria. Recognizing the inherent value in early diagnosis and intervention in SSc, researchers have endeavored to identify risk factors for progression from very early SSc to definite SSc. The present review summarizes the clinical phenotype of patients with very early and early SSc. Through a scoping review of recent literature, this review also describes risk factors for progression to definite SSc with a focus on the specific clinical features that arise early in the SSc disease course (eg, diffuse cutaneous sclerosis, interstitial lung disease, esophageal dysfunction, renal crisis, cardiac involvement). In addition to clinical risk factors, this review provides evidence for how biological data (ie, serological, genomic, proteomic profiles, skin bioengineering methods) can be integrated into risk assessment models in the future. Furthering our understanding of biological features of very early SSc will undoubtedly provide novel insights into SSc pathogenesis and may illuminate new therapeutic targets to prevent progression of SSc.Keywords: systemic sclerosis, scleroderma, early diagnosis, disease progression
