Systemic Sclerosis Without Raynaud Phenomenon in Children: The Fibrotic Subtype
Authors' conclusion
This proof-of-concept study describes a potential new clinical phenotype, termed fJSSc, characterized by predominant skin involvement, absence of SSc-specific autoantibodies, and favorable outcome.
Study context
- Population
- Consecutive patients with juvenile-onset SSc diagnosed since 2004, single centre, ≥4 years of follow-up≥4 years follow-up
- Participants
- 38 patients
- Exposure
- JSSc without Raynaud phenomenon (RP) vs JSSc with RP
- Fibrotic JSSc (fJSSc)n = 5
- RPn = 32reference
- Outcome
- Outcome: clinical remission (CR), CR on medication (CRM), or active/progressive disease (AP)
- Follow-up
- Last visit
Methodological detailssingle-center, consecutive patients, hypothesis-generating, proof-of-concept
- Single-center
- Consecutive patients
- Hypothesis-generating
- Proof-of-concept
Main finding
As reported in the abstract
Outcome in fJSSc at last follow-up
fJSSc with AP
0
fJSSc in clinical remission (CR)
3
fJSSc in clinical remission on medication (CRM) for mild pulmonary disease
2
Observational data: associations, not effects.
Further findings
Presentation with RP
Clinical features of fJSSc
- Gastrointestinal involvement
- 5
- Pulmonary involvement
- 2
- ANA positive
- 5
- SSc-specific autoantibodies
- 0
- SSc-associated autoantibodies
- 2
- SSc pattern on nailfold videocapillaroscopy
- 4/5
- Predominant skin involvement and mild internal organ involvement
Original publication
Systemic Sclerosis Without Raynaud Phenomenon in Children: The Fibrotic Subtype. J Rheumatol. Published 15 March 2026. doi:10.
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