Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease: Results of ten years of prospective surveillance

Abstract

Objective. To assess the early signs, risk factors, and rate of transition from primary Raynaud's phenomenon (primary RP) to secondary RP.Methods. A clinical sample of 307 consecutive patients with RP was included in a prospective followup study. After an initial screening, 244 patients were classified as having primary RP, of whom 236 were followed up for a mean ؎ SD of 11.2 ؎ 3.9 years. Patients classified according to the screening as having suspected secondary RP underwent an extended screening program annually until transition to secondary RP occurred.Results. The initial prevalence of secondary RP was 11%. The annual incidence of transition to suspected secondary RP was 2%, and the annual incidence of transition to secondary RP was 1%. Overall, 46 patients were classified as having suspected secondary RP, and 23 of these later were classified as having secondary RP. Older age at onset of RP (hazard ratio 2.59, 95% confidence interval [95% CI] 1.40-4.80), shorter duration of RP at enrollment (hazard ratio 0.87, 95% CI 0.81-0.94), and abnormal findings on thoracic outlet test (hazard ratio 2.69, 95% CI 1.12-6.48) were associated with an increased risk for transition to secondary RP. Compared with patients with suspected secondary RP, those diagnosed as having secondary RP had a higher number and earlier occurrence of pathologic findings. Furthermore, antinuclear antibodies at a titer of >1:320 and positive findings in specific serologic subsets were associated with a significantly increased risk for developing a connective tissue disease.

Identification of transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon by nailfold videocapillaroscopy: Comment on the article by Hirschl et al

“…In the study by Hirschl et al (1), the initial prevalence of secondary RP following the extended screening program was 11%; however, during the followup period of 11.2 Ϯ 3.9 years, the prevalence shifted to 14.9%. In our investigation, following careful analysis by nailfold videocapillaroscopy, a similar prevalence of secondary RP (14.6%) was identified, but after a much shorter followup period (29.4 Ϯ 10 months).…”

“…We read with interest the report by Hirschl et al on transition from primary to secondary Raynaud's phenomenon (RP) identified by diagnosis of an associated disease (1). The generally accepted criteria for primary RP include symmetric attacks; absence of tissue necrosis, ulceration, or gangrene; absence of a secondary cause (based on medical history and physical examination); absence of antinuclear antibodies; normal erythrocyte sedimentation rate; and, of interest, presence of normal nailfold capillaries (2).…”

The role of nail-videocapillaroscopy in early diagnosis of scleroderma

“…Other studies reported that 15-20% of patients initially diagnosed as affected by primary RP transitioned to secondary RP over a mean follow-up of 2 years [31] and [32].…”

Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud's phenomenon to systemic sclerosis: A twenty‐year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis

“…Occurring in more than 90% of patients, RP often precedes skin and visceral fibrosis by years or decades (2). Hence, at initial presentation of RP, there is a need for novel predictors to help physicians in identifying the patients who are at risk of developing SSc or another connective tissue disease (CTD) (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22).…”