Incidence of scleroderma spectrum disorders in Slovenia

Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):8-11. Epub 2012 Nov 28.


Objectives: This paper aims to investigate the incidence of scleroderma spectrum disorders (SDS) in Slovenia.

Methods: From 01.01.2007 to 31.12.2009 we prospectively examined all patients over 18 years of age suspected of suffering from SDS who were referred to our department, which is the only Rheumatology referral centre in the Ljubljana region serving a population of 518.921 Caucasians over 18. Patient work-up consisted of clinical assessment, laboratory and imaging studies, and functional tests. The working classification of SDS proposed by Maricq and Valter was used to classify patients as having CREST syndrome, digital scleroderma disease (SD), intermediate SD, diffuse SD, undifferentiated connective tissue disease with SD features, and SD sine scleroderma. Patients with certain features of SDS who did not fit any specific class were classified as having prescleroderma using LeRoy's classification of early systemic sclerosis.

Results: We examined 100 patients. Forty-one new cases of SDS were diagnosed (37 females, 4 males), aged 58.9±15.1 years (24-86 years).

Conclusions: The overall age-adjusted annual incidence of SDS in Slovenia is 2.6 per 100.000 adults per year (95%CI=1.7-3.5).

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • CREST Syndrome / epidemiology*
  • CREST Syndrome / pathology
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Prospective Studies
  • Scleroderma, Diffuse / epidemiology*
  • Scleroderma, Diffuse / pathology
  • Scleroderma, Systemic / epidemiology*
  • Scleroderma, Systemic / pathology
  • Slovenia / epidemiology
  • Young Adult