Background: Because the natural history of primary Raynaud phenomenon (RP) is unclear, we undertook this long-term (14 years) follow-up of an epidemiologic study on RP to investigate the incidence, remittance rate, and transition rate toward systemic sclerosis and other scleroderma spectrum disorders in a population-based sample of subjects.
Methods: In 1988 and 1989, 296 subjects obtained from a random sample of the general population of the Alpine valley of Tarentaise (southeast France) completed a cross-sectional study on RP. Of these, 78 met the diagnostic criteria for RP (RP+). From April 2002 to March 2003, we were able to get follow-up information on 292 people (dropout rate, 1.4%). Eighteen subjects (6.1%) had died, and the remaining 274 were successfully contacted. They were first evaluated by a standardized phone interview regarding their cold sensitivity, digital color changes, and RP. If any significant medical changes related to RP and/or suggesting scleroderma were reported, these subjects were invited for a medical evaluation.
Results: Mortality was similar in RP+ and RP- subjects, and no death was due to an RP-related condition. Seven cases of new RP were diagnosed in the RP- group, which corresponds to an annual incidence rate of 0.25%. Among the 72 RP+ subjects and the 7 subjects with a new RP available for follow-up, none developed clinical features of scleroderma. A disappearance of RP attacks for 2 winters or more was reported by 24 RP+ subjects (33%).
Conclusions: These results show that, in the general population, RP is most often a benign condition and may disappear in a substantial proportion of subjects.