Long-term outcome of Raynaud's syndrome in a prospectively analyzed patient cohort

Purpose: Knowledge of the long-term clinical outcome of Raynaud's syndrome (RS), essential both for patient counselling and formulation of optimal therapeutic recommendations, is conspicuously deficient in current medical literature. We have prospectively monitored 1039 patients with RS, 118 (11.4%) for more than 10 years to determine whether initial characterization was able to predict outcome. Methods: At initial presentation, patients were divided into four groups on the basis of vascular laboratory and serologic testing results: vasospastic, serologically positive (spast,sero+) and negative (spast,sero-) and obstructive, serologically positive (obst,sero+) and negative (obst,sero-). Results: Connective tissue disease (CTD) was present initially in 48.6% of patients with spast,sero+ results and 72.9% of patients with obst,sero+ results. Of the remaining patients in these groups, progression to CTD during follow-up occurred in 16.4% of patients with spast,sero+ results and 30.4% of patients with obst,sero+ results. In the >10-year follow-up group, progression to CTD occurred in 81.8% of patients in the obst,sero+ group. Progression to CTD occurred in 2.0% of patients in the spast,sero-group and 8.5% of patients in the obst,sero- group. Digital ulcers occurred in 15.5% of patients in the spast,sero+ group, 5.2% of patients in the spast,sero- group, 55.6% of patients in the obst,sero+ group, and 48.2% of patients in the obst,sero- group. Digital or phalangeal amputations were required in 1.4%, 1.6%, 11.6%, and 19.0% of these patients, respectively. Conclusions: The long-term outcome of patients with RS can be predicted by initial serologic studies and separation into vasospastic and obstructive categories. Initial serologic positivity strongly predicts the development of CTD. Initial vascular laboratory classification of obstructive RS strongly predicts digital ulcerations, which occurred in half of these patients regardless of initial serologic study results. Amputations were required in 10% to 20% of patients with obstructive RS. These occurrences did not increase with increased duration of disease. Ulcerations and amputations were rare in patients initially with vasospastic RS.