Clinical spectrum of Raynaud's phenomenon in patients referred to vascular clinic

Abstract

Difficulties to establish general characteristics of patients with Raynaud's phenomenon, especially frequency, rates and predisposing factors of the evolution of primary to secondary cases probably originate from substantial variation of evaluated cohorts. We conducted a prospective study using standardised diagnostic procedures in order to look for the specificity of patients referred to the vascular centre; moreover, we assayed anticardiolipin antibodies in these patients using double ELISA and compared its frequency to sex and age matched a control group of 50 healthy individuals. 124 patients (20 men), mean age at onset 35.5 yr, range 9–69 yr, had confirmed diagnosis of Raynaud's phenomenon. Ninety nine patients were found to have secondary phenomenon, 72% of them had trophic changes of fingers and/or toes. Anticardiolipin antibodies assay was positive in seven patients and four healthy donors. Vascular diseases constituted about 20%, and connective tissue diseases 50% of secondary cases, but SLE (17 cases) not a scleroderma (11 cases) was the most frequent clinical entity in the latter group. There were only two patients with Buerger's disease and one with atherosclerosis as an underlying disease for vasospastic disorder. We concluded in the vascular medicine centre that there were a lot of patients with ischemic necrosis or other type of trophic changes, and very little primary, benign Raynaud's disease cases; surprisingly, peripheral arterial occlusive disease was very seldom responsible for vasospastic episodes. Primary or secondary antiphospholipid syndrome is not associated with Raynaud's phenomenon.

Introduction

Recurrent episodes of the discoloration of fingers and/or toes brought about by reversible vasospasm when it occurs as an isolated symptom are considered primary, but in association with another disease or condition are named secondary Raynaud phenomenon (RP). While making the diagnosis of RP based on history and physical examination, particularly the observation of spontaneous or provoked sequence of skin colour changes is not difficult, the differentiation of primary from secondary RP could be laborious. Clinical or laboratory findings that can predict development of a secondary disease, mainly connective tissue disease (CTD), in initially recognised primary RP have been explored yet not fully identified 1, 2, 3, 4, 5, 6. Also, the frequency, rates, and types of diseases that evolve in these patients are not efficiently determined 7, 8, 9. All these difficulties can originate from substantial variation in the sex, ethnicity, age at entry, length of follow-up, and differences in assessment protocols. Especially, population and referral bias is likely to be significant. For example, a high percentage of scleroderma in the group of secondary RP could be at least partially influenced by the fact that most clinical data on RP come from immunology or rheumatology specialty centres, several with known interests in systemic sclerosis [10].

In order to look for the clinical characteristics of patients with RP referred to the tertiary care vascular medicine (angiology) centre we conducted a prospective study using standardised protocol containing a questionnaire for history, physical examination and established laboratory diagnostic procedures. Moreover, we evaluated the incidence of anticardiolipin antibodies in the patients with the diagnosis of RP.