Abstract
Objective
To clarify those clinical characteristics dependent on patient age at onset of Japanese systemic sclerosis (SSc).
Methods
A total of 329 SSc patients treated at Kanazawa University Hospital were enrolled in the study and subsequently categorized into four subgroups depending on age at SSc onset: childhood-onset, young-onset, normal age-onset, and late-onset. The clinical features at the first visit were compared between groups. Factors that correlated with age at onset were also analyzed.
Results
The childhood-onset subgroup was characterized by a higher male:female ratio, higher anti-topoisomerase I antibody (Ab) and anti-U3 RNP Ab levels, a higher frequency of pitting scars, and a lower frequency of anticentromere Ab and interstitial lung disease (ILD). The young-onset patients had an increased frequency of anti-U1 RNP Ab and anti-U3 RNP Ab, overlap with other connective tissue diseases, digital ulcers, and pitting scars and a reduced frequency of anticentromere Ab. Patients with late-onset SSc showed a shorter disease duration and an increased frequency of anti-RNA polymerase Ab. Multiple regression analysis showed that anti-topoisomerase I Ab, anti-U1 RNP Ab, pitting scars, and long disease duration were associated with onset at a younger age, whereas anticentromere Ab and ILD were associated with onset at an older age.
Conclusions
The clinical phenotype of SSc in Japanese patients showed a tendency to be dependent on age at onset.
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Acknowledgments
This work was supported by funds for research on intractable diseases from the Ministry of Health, Labor, and Welfare of Japan.
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M. Hasegawa and Y. Hatta contributed equally to this manuscript.
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Hasegawa, M., Hatta, Y., Matsushita, T. et al. Clinical and laboratory features dependent on age at onset in Japanese systemic sclerosis. Mod Rheumatol 23, 913–919 (2013). https://doi.org/10.1007/s10165-012-0764-0
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DOI: https://doi.org/10.1007/s10165-012-0764-0