Scleroderma epidemiology

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Incidence and prevalence

Table 1 lists U.S. studies that reported SSc incidence, prevalence, and sex distribution over a 44-year period (1947 through 1991). The Medsger and Masi study [5] was based on 86 hospitalized cases from 1947 to 1968; they derived, rather than measured, the prevalence estimate by multiplying the incidence rate (number of new cases per year) by survival in years. The Michet et al study [7] was based on 13 cases that were identified between 1950 and 1979 in Rochester, Minnesota. The Steen et al

Sex differences

As noted in Table 1, Table 2, female-to-male ratios differ somewhat among the studies but a female excess was a consistent finding. Steen et al [9] found that the excess of female cases compared with male cases is more marked in the child-bearing years compared with older age groups (3.8:1 vs. 2.4:1). This difference, however, is not as great as in SLE, where the incidence rate for women of child-bearing age is six- to ten-fold greater than that of similarly aged men [7], [25]. The implication

Racial and ethnic factors

Several United States studies have suggested that black patients have a higher age-specific incidence rate and more severe disease than white patients [9], [30]. Fig. 1, Fig. 2 show age-specific incidence rates of diffuse and limited SSc in 398 Michigan women by race for the period 1985 through 1991.

Figure 1 shows a peak age-specific incidence (20/million/year) of diffuse disease in black women in the 35- to 44-year age group. This is higher and earlier than the peak age-specific incidence of

Familial scleroderma

Some racial and ethnic groups have an increased incidence of SSc which implies that there may be a shared genetic predisposition. There have been numerous case reports of SSc occurring in more than one family member [39], [40], [41], [42], [43], [44], [45], [46], [47], [48], [49], [50], [51], [52], [53], [54], [55], [56], [57], [58], [59], [60], [61], [62], [63], [64], [65], [66], [67], [68], [69], [70], [71], [72], [73], [74], [75] (for review see [76]). Two population studies investigated

Survival

Improved survival rates were reported in recent studies [30], [83], [84], [85], [86], [87], [88] compared with earlier ones [6], [89]. This improvement is apparent and real. Earlier diagnosis is a factor that artifactually prolongs observed survival. Additionally, survivor bias, in which the rapidly progressive and fatal diffuse cases are usually not captured at the same frequency as the more mild limited cases with protracted courses, prejudices studies in the direction of increased survival.

Geographic clusters

There have been several reports of clustering of SSc in particular areas. A cluster can be defined as a greater than expected occurrence of disease cases, in a geographically defined region, that is unlikely to have resulted by chance alone [92]. Two elements are necessary in determining a cluster: (1) the underlying disease prevalence and incidence rates should be known with some reliability; and (2) the boundaries of the geographic area must reasonably conform to natural population patterns,

Summary

Evidence from multiple sources indicates that SSc does not occur randomly in the population; there are particular groups who are at greater risk. The overall incidence rate of SSc in the adult population of the United States is approximately 20 per million per year; this rate has increased from 1944 to 1973, but has been relatively stable since that time. The prevalence of SSc in the United States also seems to be stable over the past two decades with a prevalence estimate for adults of 240 per

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