Systemic sclerosis & Raynaud's
Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology
An erratum to this article was published (RMD Open, Jun 2023 ↗)
Authors' conclusion
The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc.
Study context
- Population
- Systemic sclerosis (SSc) patients from the Italian Systemic sclerosis PRogression INvestiGation registry
- Participants
- 1,808 patients
- Exposure
- Systemic sclerosis sine scleroderma (ssSSc) vs lcSSc and dcSSc
- ssSScn = 61
- Outcome
- Demographic, clinical and laboratory features of ssSSc
Methodological detailsmulticentre, national registry (SPRING)
- Multicentre
- National registry (SPRING)
Main finding
As reported in the abstract
| Group | Value | F/M |
|---|---|---|
| ssSSc | 3.4% | 19/1 |
ssSSc
Observational data: associations, not effects.
Further findings
Anticentromere positivity — ssSSc vs dcSSc
P < 0.001
Antitopoisomerase positivity — ssSSc vs dcSSc
P < 0.001
| Outcome | ssSSc | lcSSc | dcSSc | P value |
|---|---|---|---|---|
| Time from RP onset to diagnosisLonger in ssSSc than lcSSc and dcSSc | 3 (IQR 1–16.5) years | 2 (IQR 0–7) years | 1 (IQR 0–3) year | P < 0.001 |
| Digital pitting scars, ssSSc vs lcSSc | 19.7% | 42% | — not stated in the abstract | P = 0.01 |
| Digital ulcers, ssSSc vs dcSSc | 6.6% | — not stated in the abstract | 35.7% | P < 0.001 |
| Oesophageal involvement, ssSSc vs dcSSc | 46.2% | — not stated in the abstract | 63.5% | P = 0.009 |
| DLCO, ssSSc vs dcSScMean | 72.2 (SD 19.6) | — not stated in the abstract | 62.4 (SD 22.8) | P = 0.009 |
| FVC, ssSSc vs dcSScMean | 105.6 (SD 21.7) | — not stated in the abstract | 89.2 (SD 20.9) | P < 0.001 |
| Videocapillaroscopic late pattern, ssSSc vs dcSSc | 8.6% | — not stated in the abstract | 47.6% | P < 0.001 |
— not stated in the abstract
Digital pitting scars, ssSSc vs lcSSc
- Clinical ssSSc phenotype otherwise comparable to lcSSc
Anticentromere positivity
- ssSSc comparable to lcSSc
Antitopoisomerase positivity
- ssSSc comparable to lcSSc
Original publication
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