Clinical features of 405 Japanese patients with systemic sclerosis

Abstract

We aimed to clarify the clinical features of Japanese patients with systemic sclerosis (SSc), especially with reference to organ involvement and autoantibodies. A cohort of 405 patients with SSc who attended our institution from 1973 to 2008 was identified retrospectively. Data on clinical features, including autoantibodies, organ involvement, and overlap of other connective tissue diseases, were obtained by following the medical records until 2009. The percentage of male patients during or after 1990 was greater than that before 1990 (3.9 vs. 10.6%, respectively). Limited cutaneous SSc (lSSc) was twice as frequent as diffuse cutaneous SSc (dSSc). About half of the patients had lung involvement (50.4%), while only 3.2% had scleroderma renal crisis. Male gender was associated with lung involvement, and dSSc was associated with most organ involvements except for pulmonary arterial hypertension (PAH). Anti-Scl-70 antibody was associated with lung or heart involvement, while anti-U1-RNP antibody was only associated with PAH. Conversely, patients with anti-centromere antibody had less organ involvement. SSc-Sjögren overlap syndrome was related to lSSc, further overlapping systemic lupus erythematosus (SLE), and less lung or heart involvement. In conclusion, these results not only confirmed previous reports but revealed several other findings, such as the increased proportion of male patients in recent years and the relationships between clinical features.

Clinical characteristics of scleroderma overlap syndromes: comparisons with pure scleroderma

“…ATA is reported as a SSc‐specific antibody, particularly in the dcSSc subset . Our study revealed that the presence of ATA was strongly associated with pure scleroderma and the finding was supported by our subgroup analysis.…”

Clinical and autoantibody profile in systemic sclerosis: baseline characteristics from a West Malaysian cohort

“…However, the phenotypes of SSc appeared to be different in various regions of Asia. Studies in West Malaysia, India and Japan have reported more cases of lcSSc . In contrast, dcSS appears to be more commonly reported in East Malaysia, similar to the findings in Thailand, Singapore, Taiwan and China .…”

Clinical differences between Thai systemic sclerosis patients with positive versus negative anti‐topoisomerase I

“…There was a high prevalence of ATA positivity in our SSc patients (85%) compared to previous studies on Caucasians (13–50%), African‐Americans (31%) and other Asian populations (11–70%) . This might be explained by the high prevalence of the dcSSc subset among Thai SSc patients against some Asian and Western series, perhaps because of ethnic differences or genetic factors .…”

“…Anti‐U1‐RNP and anti‐PM‐Scl autoantibodies associated with SSc overlap with other connective tissue diseases, are reportedly associated with the lcSSc subset . Anti‐U1‐RNP was identified as a co‐existing antibody with ATA in a study which included SSc overlap with other connective tissue diseases . According to our study, there was no association between the presentation of ATA and the SSc subset after excluding SSc overlap with other connective tissue diseases.…”

“…Anti‐topoisomerase I antibody is a specific antibody for SSc, strongly associated with the dcSSc subset . However, it was not as strongly associated with a SSc subset among our patients after multivariate analysis.…”