Vol.23 No.5

Original Article

Clinical and laboratory features dependent on age at onset in Japanese systemic sclerosis

Authors

Minoru Hasegawa¹ , Yuri Hatta¹ , Takashi Matsushita¹ , Yasuhito Hamaguchi¹ , Manabu Fujimoto¹ , Kazuhiko Takehara¹

Received:

29 June 2012

Accepted:

28 August 2012

Published online:

19 September 2012

Full Text

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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 ageonset,
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.

Key words

Systemic sclerosis, Age at onset, Clinical features, Laboratory features