Coexistence of systemic and localized scleroderma: a systematic literature review and observational cohort study

Rheumatology (Oxford). 2020 Oct 1;59(10):2725-2733. doi: 10.1093/rheumatology/keaa297.


Objective: SSc and localized sclerosis (LoS) are considered clinically distinct entities. We describe herein the coexistence of SSc and LoS by both a systematic literature review and an observational cohort study of unselected SSc patients.

Methods: Original studies documenting the coexistence of SSc and LoS were identified in three electronic databases by means of a systematic literature search according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Additionally, the coexistence of SSc and LoS was studied in a prospective cohort of SSc patients visiting the Ghent University Scleroderma Unit for their yearly follow-up visit between January 2018 and January 2019.

Results: Five studies were finally included for quality appraisal and data extraction. The coexistence of SSc and LoS ranged between 2.4 and 7.4%. RP, scleroderma pattern on nailfold videocapillaroscopy (NVC) and the presence of SSc-specific antibodies were commonly observed in coexistent cases. Additionally, coexistence of SSc and LoS was found in 8/296 (2.7%) consecutive SSc patients of the Ghent University Scleroderma Unit. RP was present in 6/8 coexistent cases; a scleroderma pattern on NVC was observed in all coexistent cases, and SSc-specific antibodies (i.e. cenp-B) were found in 4/8 coexistent cases.

Conclusion: This is the first systematic literature review with additional cohort evaluation investigating the coexistence of SSc and LoS. A relatively high overlap of SSc and LoS was revealed, which is peculiar because both are rare diseases.

Keywords: coexistence; localized scleroderma; systematic literature review; systemic sclerosis.

Publication types

  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Adult
  • Aged
  • Antibodies / blood*
  • Antibodies / immunology
  • Case-Control Studies
  • Cohort Studies
  • Databases, Factual
  • Female
  • Humans
  • Male
  • Microscopic Angioscopy / methods*
  • Middle Aged
  • Nails / diagnostic imaging*
  • Nails / pathology
  • Observational Studies as Topic
  • Prevalence
  • Scleroderma, Localized / complications*
  • Scleroderma, Localized / diagnosis
  • Scleroderma, Localized / epidemiology
  • Scleroderma, Localized / immunology
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / epidemiology
  • Scleroderma, Systemic / immunology


  • Antibodies