Epidemiological study of patients with systemic sclerosis in Tokyo

Tamaki, Takeshi; Mori, Shunji; Takehara, Kazuhiko

doi:10.1007/bf00371817

Cited by 118 publications

(62 citation statements)

References 9 publications

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“…In 120 patients, including all 9 patients with morphea, antinuclear antibody (ANA) test was performed using inch-rect immunofluorescence and double immunodiffusion methods, as previously described [7,8], Characteristics of SSc patients with and without morphea arc summarized in tabic I. That only 5 patients were male is consistent with the recent reports of a very low male to female ratio [9,10].…”

Section: Methodssupporting

confidence: 68%

Coexistence of Morphea and Systemic Sclerosis

et al. 1993

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While the association of morphea and systemic sclerosis (SSc) is considered to be a rare condition, we observed well-demarcated sclerotic skin changes indistinguishable from morphea in 9 of 135 SSc patients who visited our clinic during the last decade. We consider this rate of incidence (6.7%) to be high enough to consider morphea to be one of the skin involvements of SSc. There was a significantly (p < 0.01) higher incidence of morphea in males (3 of 5) than in females (6 of 130). Only 3 of Ill SSc patients positive for antinuclear antibody (ANA) also showed morphea, whereas 6 of 9 patients negative for ANA showed morphea (p < 0.01). Although the mechanism underlying the development of morphea in SSc patients remains unknown, our observations suggest a heterogeneous pathogenesis related to SSc gender and ANA type.

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Section: Methodssupporting

confidence: 68%

Coexistence of Morphea and Systemic Sclerosis

et al. 1993

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“…However, we found a statistically significant increase in the percentage of males with SSc in our more recent cohort, from 3.9% (up to 1989) to 10.6% (from 1990 onward). Indeed, a previous Japanese study of SSc in patients with the onset before 1988 also showed a low percentage of males (6.4%, n = 636) [21]. This supports the hypothesis that the sex ratio of Japanese SSc patients has been changing recently with an increase in the number of male patients.…”

Section: Discussionmentioning

confidence: 52%

Clinical features of 405 Japanese patients with systemic sclerosis

et al. 2011

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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.

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“…In England, the prevalence of SSc has been reported as 30.8 cases per million (based on 156 cases) (34), in Japan the prevalence estimate is 38 cases per million (based on 357 cases) (35), and in Iceland the prevalence estimate is 71 cases per million (based on 37 cases) (36). These studies included both limited and diffuse forms of SSc.…”

Section: Discussionmentioning

confidence: 99%

Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population

Mayes¹,

Lacey²,

Beebe‐Dimmer³

et al. 2003

Arthritis & Rheumatism

835

491

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Objective. To estimate the prevalence, incidence, survival, and disease characteristics of systemic sclerosis (SSc) in the Detroit tricounty area.Methods. A census of SSc cases for the period 1989-1991 was conducted in the Detroit area, using multiple sources for case identification. Diagnoses were verified by medical record review. Capture-recapture analysis was used to estimate the total SSc population. Cases of localized scleroderma (morphea and linear disease) were excluded.Results. Based on 706 verified cases of SSc, prevalence was initially estimated to be 242.0 cases per million adults (95% confidence interval [95% CI] 213-274), with an annual incidence of 19.3 new cases per million adults per year (95% CI 12.4-30.2). Capturerecapture analysis, based on the degree of overlap of verified cases among multiple sources, resulted in a revised prevalence estimate of 276 cases per million adults (95% CI 245-310). Sex-and race-specific prevalence estimates were significantly higher for women than for men, and for blacks than for whites. The average age at diagnosis was significantly younger for blacks than for whites. Compared with white patients, black patients were almost twice as likely to have diffuse disease (prevalence proportion ratio 1.86, 95% CI 1.48-2.35). Median survival was ϳ11 years. Factors negatively affecting survival included male sex (hazard ratio 1.81, 95% CI 1.29-2.55) and older age at diagnosis (hazard ratio 1.04, 95% CI 1.03-1.05).Conclusion. This study establishes baseline estimates of SSc occurrence and characteristics in a large US cohort consisting primarily of black adults and white adults. These data should facilitate research regarding the role of geographic, ethnic, racial, and environmental factors for this disease in comparison populations.

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Epidemiological study of patients with systemic sclerosis in Tokyo

Cited by 118 publications

References 9 publications

Coexistence of Morphea and Systemic Sclerosis

Coexistence of Morphea and Systemic Sclerosis

Clinical features of 405 Japanese patients with systemic sclerosis

Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population

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