Utako Kaneko, Takako Miyamae, Yasuhito Hamaguchi, Masaki Shimizu, Ikuko Ueda-Hayakawa, Hideki Ishikawa, Ryusuke Ae, Yoshikazu Nakamura, Yoshihide Asano, Yasushi Kawaguchi, Masataka Kuwana, Daisuke Goto, Minoru Hasegawa, Masaru Hatano, Yohei Isomura, Masatoshi Jinnin, Yasuhiro Kanatani, Atsushi Kumanogoh, Takamitsu Makino, Katsunari Makino, Takashi Matsushita, Sei-ichiro Motegi, Naoki Mugii, Naoko Okiyama, Yoshihito Shima, Hiroki Takahashi, Sumiaki Tanaka, Toshiyuki Yamamoto, Hidekata Yasuoka, Hisataka Maki, Ayumi Yoshizaki, Manabu Fujimoto and Ministry of Health, Labour and Welfare’s Systemic Sclerosis Research Group
Niigata University Graduate School of Medical and Dental Sciences. Tokyo Women’s Medical University Hospital. Kanazawa University. Graduate School of Medical and Dental Sciences, Institute of Science Tokyo. University of Osaka Graduate School of Medicine. Kyoto Prefectural University of Medicine. Jichi Medical University. Tohoku University Graduate School of Medicine. Tokyo Women’s Medical University School of Medicine. Nippon Medical School Graduate School of Medicine. Ibaraki Prefectural Central Hospital and Cancer Center. University of Fukui. University of Tokyo Hospital. Wakayama Medical University. Tokai University. Kumamoto University Faculty of Life Sciences School of Medicine. Gunma University Graduate School of Medicine. Sapporo Medical University School of Medicine. Fukushima Medical University. Fujita Health University.
Japan
Journal of Rheumatology
J Rheumatol 2025;
DOI: 10.3899/jrheum.2025-0175
Abstract
Objective: To evaluate clinical and epidemiological features of juvenile-onset systemic sclerosis (jSSc) in Japan and to identify racial and generational differences.
Methods: We surveyed patients with jSSc (developed before the age of 18 years) who visited selected facilities in Japan between January 2016 and December 2020. We estimated the number of patients with jSSc and the annual incidence rate in Japan. Thereafter, differences in clinical characteristics by disease subtype, autoantibody, and age at investigation were analyzed and compared with previous cohorts.
Results: Of the 3,005 institutions selected for the first survey, 1,845 (61.8%) responded. The estimated number of patients with jSSc was 299, while the estimated annual incidence rate ranged from 0.98 to 1.59 per 1,000,000 children (aged <18 years) from 2016 to 2020. In the second-stage survey, 130 cases were analyzed, of which 85 (65.4%) had diffuse cutaneous SSc (dcSSc), 77.7% were female, while the median ages at onset and survey was 11 and 21 years, respectively. Autoantibody positivity was 62.4% for anti-topoisomerase I antibody (ATA) and 12.9% for anti-centromere antibody, while anti-PM-Scl antibody was very rare. In total, interstitial lung disease was present in 40.8% of patients (predominantly dcSSc and ATA positive), gastrointestinal lesions in 36.9%, pulmonary arterial hypertension in 7.7%, and no renal crisis.
Conclusion: This is the largest national survey of jSSc characteristics analyzed in detail by autoantibody and disease subtype. Japanese jSSc was characterized by very high ATA-positivity rate. However, the frequency of major organ involvement was similar to previous reports of jSSc in the west.
Category
Class I. Pulmonary Hypertension Associated with Connective Tissue Disease
Symptoms and Findings Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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