Clinical characteristics of primary, secondary and suspected secondary Raynaud’s syndrome and diagnostic transition in the long-term follow-up. A retrospective study in 900 patients

Abstract

Background: There have so far been only partial studies or studies in fairly small groups of Raynaud’s patients to systematically analyse the clinical features, the beginning of first Raynaud’s attacks, the interval between first Raynaud’s attacks and the first clinical examination, the duration of Raynaud’s attacks, inducing factors, frequency of attacks, localization, coloration, accompanying symptoms, laboratory parameters, instrument-based examinational findings on the first examination, fitness for work, and development of the diagnostic classification in the long term. Such studies are, however, necessary for reasons of differential diagnostics and therapeutics. Patients and methods: The disease data of 900 patients with established Raynaud’s symptoms, who underwent an examination between 1973 and 2002, were at first analysed in retrospect on the basis of their case history. This was followed, in 2002 to 2003, by a mail inquiry of all 900 patients, who were sent a comprehensive questionnaire to confirm history data and supplement clinical findings. Answers were received from 419 patients. In addition, 177 of the 900 patients underwent at least one follow-up examination after 2.8 years (1 month – 18 years), on the average, which allowed checking whether and how the diagnostic classification into primary, secondary and suspected secondary Raynaud’s syndrome had changed. All data were analysed statistically. Results: Of the 900 patients, 77% were women and 23% were men. The mean age on the first examination was 45.1 ± 15.5 years. Calculated on all patients, the first Raynaud’s attacks occurred at the age of 39.5 ± 16.9 years. The beginning of Raynaud’s attacks fell mostly between the 21st and 60th year of life (minimum 3rd year, maximum 88th year). There was no statistical difference between primary, secondary and suspected secondary Raynaud’s syndromes as to the time, ranging from 38.8 to 44.3 years of age, when the symptoms began. The duration of Raynaud’s attacks averaged 23.6 ± 23.5 min.; they occurred every day in 59.1%, once to three times a day in 26.8%, and more than three times a day in 31.1% of the cases. Factors inducing Raynaud’s attacks were cold in 96.8% and stress in 19.2%. Raynaud’s phenomenon was observed in both hands in 90.9% and in one hand only in 8.6% of the cases. A white discoloration only was observed in 39.0%, a white-blue discoloration sequence, in 19.3%, a purely blue discoloration, in 7.6%, and a triphasic colour response, in 19.1%. The latter sign was significantly more frequent in women and secondary Raynaud’s syndrome than in men and primary Raynaud’s syndrome. At 64.6%, secondary Raynaud’s syndrome was significantly more often associated with pain than primary Raynaud’s syndrome (47.9%). The mean systolic blood pressure was 125.6 ± 20.9 mmHg, the mean diastolic pressure, 78.3 ± 10.6 mmHg. Concomitant diseases were equally frequent in primary, secondary and suspected secondary Raynaud’s syndrome. Finger, hand and arm artery occlusions were equally often seen in all three forms of Raynaud’s syndrome. The results of the first examination showed that 72.9% of the 900 patients suffered from primary, 5.3% from secondary, and 21.3% from suspected secondary Raynaud’s syndrome. In the long term, primary and suspected secondary Raynaud’s syndromes showed a diagnostic transition in 19.2% of the cases after 2.8 years, on the average, while secondary Raynaud’s syndromes did not. The clinical symptoms of the groups, as found on the first examination, were not altered by this transition, though. Conclusion: The clinical characteristics of Raynaud’s syndromes (age when first Raynaud’s attacks are observed, inducing factors of Raynaud’s attacks, duration frequency of attacks, localization, coloration, and accompanying symptoms of an attack) are not specific and therefore not suitable to distinguish between primary and secondary Raynaud’s syndrome. The case history, a general clinical examination, immunoserologic and capillaroscopic findings, and repeated examinations during 5 or more years are necessary to differentiate between primary, secondary and suspected secondary Raynaud’s syndromes.