Objective: To evaluate prospectively a cohort of patients with Raynaud's phenomenon (RP) and signs, symptoms, or laboratory abnormalities suggestive of a connective tissue disease (CTD) to determine prognosis and to identify predictors of evolution.
Methods: Patients with suspected secondary RP were evaluated at baseline, 2.7 years, and 8.4 years after entry by history and examination, chest radiograph and barium esophagram, pulmonary function tests, antinuclear and anticentromere antibodies (ACA), cryoglobulins, and nailfold capillary microscopy (NCM). Logistic regression was used to identify predictors of evolution and to develop a risk factor model.
Results: Sixty-four patients were entered 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 predictors of the development of systemic sclerosis. A positive ACA was the only risk factor identified for evolution into CREST syndrome (calcinosis, RP, esophageal dysmotility, sclerodactyly, telangiectasias) (OR = 22.5). Finally, nailfold capillary abnormalities were the only baseline feature associated with the development of any definite CTD (OR = 8.3).
Conclusion: Fifty percent of patients with suspected secondary RP will develop a CTD over a period of 8.4 years. NCM predicts development of systemic sclerosis or any definite CTD and should be included in the evaluation of all such patients.