Malignancies Occurring During

Case reports suggest that the development of the two diseases is not coincidental and that scleroderma patients have twice the risk of developing malignancies than the general population. Lung and perhaps breast cancers seem to be more frequent in PSS patients. In a Danish series of 344 patients [7], scleroderma was responsible for a 3-fold increased risk of death (Table 1). Most patients died of PSS-related causes (acute renal crisis, hypertension, lung fibrosis, etc.), but malignancies were also described. Lung cancer was the most frequent cause of death-related malignancies (13/160 deaths). Hematological malignancies were the second most common cause of death in the cancer group (4 cases). The other causes were anecdotal: breast and ovarian cancer (3 of each), uterus, ovarian etc. An over-representation of lymphoma has been suggested by others [8] who observed 2 cases of non-Hodgkin's lymphoma in PSS patients. These authors reported that the standardized incidence ratio for all cancers among the 233 patients was sharply increased (by 2.4). The risk of lung cancer was increased by 7.8 and the risk of non-Hodgkin's lymphoma by 9.6. Similar results had been previously published [9]. Other cases of non-Hodgkin's lymphoma have been reported [10-12] and support the association with PSS.

Other papers also described cancers occurring in PSS: esophageal cancer, particularly in patients with Barrett's syndrome [13], ovarian and breast cancers [1, 10, 14, 15]. In studies by Rosenthal et al.. [8] and Ja-cobsen et al. [7], the frequencies of breast and ovarian cancer were not higher than in the general population.

In Japan [16], the frequency of lung cancer in PSS patients suggests that race has no influence on the occurrence of the two diseases.

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