Towards the definition of disease phenotypes in paediatric SAPHO syndrome: a national multicentric study
Authors' conclusion
Our data have identified two different phenotypes of pSAPHO based on skin manifestations, with different age of onset, gender, and response to treatments. These two groups have peculiar clinical features that differ from those of the CNO group.
Study context
- Population
- Italian patients with paediatric SAPHO syndrome enrolled in the Eurofever Registry, compared with patients with chronic recurrent multifocal osteomyelitis (CRMO)54 pSAPHO with skin manifestations
- Participants
- 35 / 19 / 167
- Exposure
- pSAPHO with acne - hidradenitis suppurativa (Acne-HS) vs pSAPHO with PPP-PV vs CNO without skin manifestations
- Acne-HSn = 35
- PPP-PVn = 19
- CNOn = 167reference
- Outcome
- Clinical features by skin phenotype
Methodological detailsmulticentre, registry-based (Eurofever Registry)
- Multicentre
- Registry-based (Eurofever Registry)
Main finding
As reported in the abstract
Males in Acne-HS
82.9%
Females in PPP-PV
84.2%
Observational data: associations, not effects.
Further findings
Axial pattern — Acne-HS and PPP-PV vs CNO
P < 0.0001
Biologic therapy required
Biologic therapy required
- Acne-HS had refractory skin disease requiring steroids and other lines of treatment; PPP-PV responded well to biologics
Original publication
Towards the definition of disease phenotypes in paediatric SAPHO syndrome: a national multicentric study. Rheumatology (Oxford). Published 5 February 2025. doi:10.
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