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Validated clinimetric criteria, useful in the early phase of systemic sclerosis, are lacking
Systemic sclerosis (SSc) is a multisystemic disorder of unknown cause characterised by fibrotic and degenerative changes in skin, blood vessels and internal organs.1 The clinical spectrum of SSc, and hence its prognosis, is quite variable.2 Validated subset classification, activity and severity criteria are available.2–7 However, despite their usefulness, these criteria have several limitations. Furthermore, diagnostic criteria of SSc are lacking. To improve the care of patients with scleroderma, the clinician needs to initiate optimal, early treatment. Diagnostic criteria that aid early diagnosis as well as classification, severity criteria that aid estimation of prognosis, and disease activity criteria that indicate active disease could help the clinician in daily practice. Present international collaborations such as the EULAR Scleroderma Trial and Research Group (EUSTAR) and the North American Scleroderma Clinical Trials Consortium (SCTC) are thriving, providing the ideal circumstances for clinimetric studies of SSc.
This editorial discusses the state of the art of clinimetrics in SSc and possible ways to overcome the present limitations.
Diagnostic criteria, especially for the early phase, are indispensable for estimation of prognosis and for timely initiation of treatment. The American College of Rheumatology (ACR) Preliminary Criteria for the Classification of Systemic Sclerosis (Scleroderma), developed in 1980, are often used to diagnose patients with SSc.3 The objective of these classification criteria was to establish a standard for definite or certain disease in order to permit comparison of groups of patients from different centres and to assist in proper evaluation of the results of clinical and therapeutic trials and not to aid the diagnosis of early SSc in the individual patient.3 These preliminary criteria were established in a prospective multicentre study in which adult patients with scleroderma were included who were …