Prevalence of scleroderma spectrum disorders in the general population of south carolina

Abstract

The prevalence of scleroderma spectrum disorders (including systemic sclerosis [SSc] meeting the American Rheumatism Association criteria and the less typical disorders meeting only our study criteria) was determined in a random sample of 6,998 subjects from the general population of South Carolina. The results suggest that the prevalence of these disorders may range from 67 to 265 per 100,000, which is 4.9 to 19.2 times higher than previously reported for definite SSc. The ratio of nondefinite cases to definite cases of SSc (those meeting American Rheumatism Association criteria) was 2.5. Most of the nondefinite cases were unrecognized prior to our study, which suggests the need for improved early diagnosis of scleroderma spectrum dis- orders. Brief histories of the 7 patients with scleroderma spectrum disorders whose cases formed the basis for our calculation of prevalence rates are included in this report.

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

“…Our predicted prevalence of 242.0 cases per million adults based on our 706 verified cases is similar to the prevalence estimate of 286 cases per million reported by Maricq et al (5), based on 2 cases of definite SSc in a South Carolina population. However, the small number of cases in the Maricq study precludes much confidence in this estimate.…”

“…dence of systemic sclerosis (SSc; scleroderma) vary widely depending on the period of observation, methods of case ascertainment, and the geographic area of study (1)(2)(3)(4)(5)(6); for review, see ref. 7).…”

“…Reported US incidence rates vary between 2.7 cases per million per year for the time period 1947-1968 (2) and 18.7 cases per million per year for the time period 1972-1982 (6). Similarly, US prevalence estimates have varied from 138 cases per million for the period 1950-1979 (4) to 286 cases per million in 1985 (5). These discrepancies may reflect true variation in disease occurrence among different populations or may be related to methodologic differences, such as the degree of scrutiny applied or the classification of disease.…”

The role of nail-videocapillaroscopy in early diagnosis of scleroderma

“…Despite a large prevalence in the general population [2], Raynaud's phenomenon remains a typical manifestation of vascular involvement in scleroderma. It is characterized by intense vasospasm of the digital arteries under cold exposure or emotional stress, which leads to characteristic color changes in the skin of the fingers, which is the expression of the imbalance between vasoconstrictor and vasodilator factors [3].…”

Raynaud’s Phenomenon: A Multidisciplinary Problem

“…Так, почти 12% из 7000 человек ответили утвердительно на во-прос о повышенной чувствительности пальцев или конечностей в целом к холодной температуре [30]. Более того, холодная кожа или неочерченная крапчатая окраска кожи пальцев, кистей и конеч-ностей считается нормальным ответом на воз-действие холода.…”