Clinical and epidemiological features of juvenile-onset systemic sclerosis from a nationwide survey in Japan
Authors' conclusion
Japanese jSSc was characterized by a very high ATA positivity rate. However, the frequency of major organ involvement was similar to previous reports of jSSc in the West.
Study context
- Population
- Patients with juvenile-onset SSc (developed <18 years of age) visiting selected facilities in Japan, January 2016–December 2020second-stage survey cases analysed
- Participants
- 130 patients
- Outcome
- Estimated number of patients with jSSc and annual incidence rate in Japan
Methodological detailsnationwide survey, two-stage survey
- Nationwide survey
- Two-stage survey
Main finding
As reported in the abstract
Estimated number of patients and annual incidence of jSSc in Japan · 2016 to 2020
Estimated number of patients with jSSc
299
Estimated annual incidence rate per 1 million children (aged <18 years)
0.98 to 1.59
Observational data: associations, not effects.
Further findings
Clinical characteristics (second-stage survey, 130 cases analysed)
- Diffuse cutaneous SSc (dcSSc)
- 85 (65.4%)
- Female
- 77.7%
- Median age at onset, years
- 11
- Median age during survey, years
- 21
Autoantibody positivity
- Antitopoisomerase I antibody (ATA)
- 62.4%
- Anticentromere antibody
- 12.9%
- Anti-PM/Scl antibody very rare
Organ involvement
- ILD (predominantly dcSSc and ATA positive)
- 40.8%
- Gastrointestinal lesions
- 36.9%
- PAH
- 7.7%
- No renal crisis
Original publication
Clinical and epidemiological features of juvenile-onset systemic sclerosis from a nationwide survey in Japan. J Rheumatol. Published 15 September 2025. doi:10.
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