Systemic Sclerosis, Ulcer
Conditions
Brief summary
Systemic sclerosis is an orphan, multiorgan disease affecting the connective tissue of the skin and several internal organs. Digital ulcers are frequent and have a major impact on the quality of life in patients with systemic sclerosis. The etiology of digital ulcers is complex and multifactorial and the principal mechanisms underlying the digital ulcers formation are ischemic, mechanic and inflammatory, alone or in combination, on the basis of the systemic sclerosis vasculopathy. Consequently, there are at least three types of DU: (i) those localized at the tips of the fingers and toes, mainly resulting from an ischemic process, (ii) those localized on the dorsal aspect of the fingers where the skin retraction due to fibrosis over bony prominences seems to be the main cause, and (iii) those evolving on a pitting scar or subcutaneous calcinosis due to a combined irritative-inflammatory mechanism. An early therapy to prevent or rapidly heal digital ulcers is today considered a mandatory approach to maintain quality of life and spare the enormous costs due to conventional digital ulcer management. This observational trial is part of the collaborative project DeSScipher, one out of five observational trials to decipher the optimal management of systemic sclerosis. Aim of this observational trial is: 1. To identify the best treatment combination for prevention of digital ulcers in patients with fulfilment of the new ACR/EULAR SSc criteria or the preliminary VEDOSS criteria for very early diagnosis of systemic sclerosis 2. To identify the best treatment associated with improved healing of digital ulcers in patients with fulfilment of the new ACR/EULAR SSc criteria Thus, the observational trial consist of a prevention arm and a healing arm.
Interventions
None listed
Sponsors
European Union
University of Giessen
University of Zurich
University of Paris 5 - Rene Descartes
University of Campania Luigi Vanvitelli
University of Basel
University College, London
Charite University, Berlin, Germany
University of Pecs
University of Leeds
Schoen Klinik Hamburg Eilbek
marco matucci cerinic
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
* Juvenile and adult patients with diagnosis of systemic sclerosis according to the ACR/EULAR SSc criteria or the PRES/ACR/EULAR juvenile SSc criteria for enrollment into the prevention and healing arm * Patients with fulfilment of the preliminary criteria for very eary diagnosis of systemic sclerosis (VEDOSS criteria) for enrollment into the prevention arm Patients with active digital ulcers will be allocated into the healing arm. Patients without previous or history of digital ulcers (but currently without active digital ulcers) will be allocated into the prevention arm. Definition of digital ulcers (DU): DU are 1) loss of tissue, 2) DU derived from digital pitting scars and 3) DU derived from calcinosis. IN THIS STUDY WE CONSIDER ONLY DU (1) LOSS OF TISSUE. THE OTHER DU (2) and DU (3) ARE REGISTERED IN THE DATA BASE IF THEY ARE PRESENT, BUT ARE EXCLUDED FROM EVALUATION.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Prevention arm: Number of new digital ulcers | 24 months |
| Healing arm: Time to healing of manifest digital ulcers | 24 months |
Secondary
| Measure | Time frame |
|---|---|
| Prevention arm: Time to development of new digital ulcers | 24 months |
| Healing arm: Number of healed digital ulcers | 24 months |
Other
| Measure | Time frame | Description |
|---|---|---|
| Prevention arm: novel composite digital ulcer prediction score | 24 months | Prevention: A novel composite digital ulcers prediction score which will be tested against the already proposed capillaroscopic prediction score CSURI |
| Healing arm: novel composite outcome score for the assessment of worsening of digital ulcers despite treatment | 24 months | Healing: A novel composite outcome score for the assessment of worsening of digital ulcers despite treatment |
Countries
France, Germany, Hungary, Italy, Switzerland, United Kingdom
Contacts
Primary ContactMarco Matucci-Cerinic, Prof.
Backup ContactFrancesco Del Galdo, Prof.
Outcome results
None listed