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Very early versus early disease: the evolving definition of the ‘many faces’ of systemic sclerosis
  1. Marco Matucci-Cerinic,
  2. Silvia Bellando-Randone,
  3. Gemma Lepri,
  4. Cosimo Bruni,
  5. Serena Guiducci
  1. Department of Rheumatology AVC, Department of Biomedicine & Division of Rheumatology AOUC, Department of Medicine & Denothe Centre, University Florence, Florence, Italy
  1. Correspondence to Professor Marco Matucci-Cerinic, University of Florence, Department of Internal Medicine, Division of Rheumatology, Villa Monna Tessa, Florence 50139, Italy; cerinic{at}

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Systemic sclerosis (SSc) is a challenge for the rheumatologist as it is easy to diagnose when it is evolved to skin fibrosis with obliterative vasculopathy and organ involvement but its diagnosis remains very difficult in the very early/early phase of the disease because the American College of Rheumatology (ACR) and LeRoy criteria, which are currently those largely used to classify SSc, were shown to be not sensitive enough to reach a very early or an early diagnosis of SSc.1–4 Therefore, the SSc diagnosis may be delayed for several years following the onset of Raynaud's phenomenon (RP) and even after the onset of the first non-RP symptom. This reality implies that the diagnosis, and consequently the therapy, are delayed until skin involvement and/or internal organ involvement are evident5–8 and, in too many cases, already irreversible.6

The fact that organ involvement may be present from the earliest stages of SSc corroborates the necessity of a very early or at least an early diagnosis of SSc to try to identify therapy which might achieve disease remission. Moreover, the rheumatologist is also facing today the problem to position the patient in the SSc evolution from its very early to the early and to the established phase. Previously, several attempts have been made to define the early phase of SSc7 and recently even the concept of very early SSc has been widely accepted and investigated.8 RP, despite its lack of specificity, has been proposed as a pivotal sign in a previous attempt to define criteria for the diagnosis of ‘early’ SSc9 and has been also considered as the main ‘sentinel’ sign10 for the identification of ‘very early …

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  • Contributors MMC conceived the study and participated in the drafting of the manuscript. SBR, GL and CB participated in the drafting of the manuscript. SG coordinated the study and participated in the drafting of the manuscript.

  • Funding This work was funded by a grant provided by the Regione Toscana, Italy (Regional Health Research Programme).

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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