OUTCOMES IN PRIMARY RAYNAUD PHENOMENON - A METAANALYSIS OF THE FREQUENCY, RATES, AND PREDICTORS OF TRANSITION TO SECONDARY DISEASES

Objective: To summarize the current literature on the frequency, rates , types, and outcome predictors of secondary diseases that develop in patients with primary Raynaud phenomenon. Methods: A structured MEDLIN E literature search with the MeSH heading ''Raynaud's disease,'' which was crossed with (1) systemic sclerosis, (2) prognosis, (3) prospecti ve studies, (4) follow-up studies, and (5) retrospective studies, was used to identify 910 articles for possible inclusion. Articles that id entified patients with primary Raynaud phenomenon who were followed up and reevaluated at the end of the study, and which used published cla ssification criteria to assess the presence or absence of secondary di sease were included. Patient-years of Raynaud disease, patient-years o f follow-up, and rates and predictors of transition to secondary disea se were calculated from the articles selected. The summary odds ratio and positive predictive value for evaluation criteria at entry were ca lculated from 2x2 tables generated for each variable. Results: Ten art icles identified a total of 639 patients with primary Raynaud phenomen on who were followed up for 2531 patient-years. Eighty-one patients (1 2.6%) developed a secondary disorder, 80 of which were connective-tiss ue diseases. Transitions occurred at a mean rate of 3.2 per 100 patien t-years of observation. The mean time to develop a secondary disorder was 2.8 years from study entry and 10.4 years from the onset of Raynau d phenomenon. At entry, the best predictor of transition was an abnorm al nailfold capillary pattern (positive predictive value, 47%). Antinu clear antibodies in these patients had a positive predictive value of only 30%. Conclusion: Although a variety of clinical and serological a bnormalities can be found in patients with primary Raynaud phenomenon, only a small percentage of them develop a connective-tissue disease.