THE EVOLUTION OF RAYNAUDS-PHENOMENON - A LONG-TERM PROSPECTIVE-STUDY

Objective. To evaluate prospectively a cohort of patients with Raynaud 's phenomenon (RP) and signs, symptoms, or laboratory abnormalities su ggestive of a connective tissue disease (CTD) to determine prognosis a nd to identify predictors of evolution. Methods. Patients with suspect ed secondary RP were evaluated at baseline, 2.7 years, and 8.4 years a fter entry by history and examination, chest radiograph and barium eso phagram, pulmonary function tests, antinuclear and anticentromere anti bodies (ACA), cryoglobulins, and nailfold capillary microscopy (NCM). Logistic regression was used to identify predictors of evolution and t o develop a risk factor model. Results. Sixty-four patients were enter ed and all were subsequently evaluated, Thirty-two (50%) progressed to a definite CTD. Abnormalities of nailfold capillaries [odds ratio (OR ) = 21.8] and hand swelling (OR = 18.5) at baseline were independent p redictors of the development of systemic sclerosis. A positive ACA was the only risk factor identified for evolution into CREST syndrome (ca lcinosis, RP, esophageal dysmotility, sclerodactyly, telangiectasias) (OR = 22.5). Finally, nailfold capillary abnormalities were the only b aseline feature associated with the development of any definite CTD (O R = 8.3). Conclusion. Fifty percent of patients with suspected seconda ry RP will develop a CTD over a period of 8.4 years. NCM predicts deve lopment of systemic sclerosis or any definite CTD and should be includ ed in the evaluation of all such patients.