Reumatología Clínica

Reumatología Clínica

Volume 15, Issue 6, November–December 2019, Pages e92-e95
Reumatología Clínica

Original Article
Retrospective view of primary Raynaud's phenomenon in childhoodRetrospectiva del fenómeno de Raynaud primario en la infancia

https://doi.org/10.1016/j.reuma.2017.12.012Get rights and content

Abstract

Objectives

Primary Raynaud's phenomenon (PRP) manifests as episodes of transient spasms of peripheral blood vessels. To elucidate the clinical clues and laboratory characteristics will facilitate the identification of PRP.

Methods

A retrospective data collection of clinical and laboratory characteristics of 58 children with PRP was performed between January 2007 and December 2016.

Results

A positive ANA test at lower titers <1:100 was detected in 24.1% of the patients. There was a significant relationship between presence of ANA positivity and migraine in female patients with PRP (p = 0.01; p = 0.020 respectively). The most common accompanying disorder was migraine which was detected in 37.9% of all patients with PRP. Hemoglobin and serum ferritin levels were significantly lower in PRP patients with migraine (p = 0.045; p < 0.05, respectively). Additionally, the mean platelet volume (MPV) measurements were significantly higher in patients with migraine compared to those without migraine (p = 0.045; p < 0.05 respectively).

Discussion

There is limited data concerning childhood PRP. For the first time we showed a high frequency of migraine in childhood PRP. Anemia and high MPV could be the underlying triggering factors of these two episodic diseases.

Resumen

Objetivo

El fenómeno de Raynaud primario (PRP, por sus siglas en inglés) se manifiesta como episodios de vasoespasmos transitorios de vasos sanguíneos periféricos. Elucidar las características clínicas y de laboratorio facilitará la identificación del PRP, así como las enfermedades acompañantes.

Métodos

Se realizó una un estudio retrospectivo de datos clínicos, de laboratorio y de tratamiento en 58 niños con PRP entre enero de 2007 y diciembre de 2016.

Resultados

Se detectó una prueba de ANA positiva en títulos inferiores (< 1:100) en el 24,1% de nuestros pacientes. Se encontró correlación estadística entre la positividad de anticuerpos antinucleares y la presencia de migraña en las mujeres con PRP (p = 0,01; p = 0,020, respectivamente, prueba exacta de Fisher, prueba de corrección de continuidad de Yates). El trastorno más común fue la presencia de migraña en el 37,9% (n = 22) asociado con PRP. Los niveles de hemoglobina fueron significativamente más bajos asociados con un bajo nivel de ferritina sérica en los casos de PRP con migraña (p = 0,045; p < 0,05). Además, las mediciones del volumen plaquetario medio (VPM) fueron significativamente mayores en los casos con migraña en comparación con aquellos sin migraña (p = 0,045; p < 0,05).

Discusión

Existen datos muy limitados sobre el PRP infantil. Hemos presentado por primera vez la asociación de PRP infantil y migraña en un estudio retrospectivo. La anemia y el VPM alto podrían formar parte de los factores predisponentes de ambas enfermedades episódicas.

Introduction

Raynaud's phenomenon (PRP) is a vascular disorder characterized by episodic and reversible attacks of vasospasm with paleness of the extremities, followed by cyanosis and hyperemia. Arteriovenous anastomoses are richly innervated by sympathetic nerves and are normally exposed to increased sympathetic vasoconstriction under resting thermoneutral conditions and when sympathetic activity is increased during stress or exposure to cold. Prevalence of PRP in the general population ranges 5–20%, while in between 12 and 15 year old children this rate is 15%.1, 2, 3, 4 Females are much more susceptible to RP than males. Hormonal factors may account for the higher prevalence of RP in females. Primary RP occurs without any underlying disease and is considered a benign condition that is mostly seen during childhood. In contrast, secondary RP is associated with other diseases; mainly connective tissue diseases. The clinical absence of any underlying disease, negative autoimmune serology and serum inflammatory markers associated with normal capillaroscopy findings lead to the diagnosis of PRP.

A significant increase has been reported in the prevalence of migraine in adult patients with PRP and vice versa.5, 6 Vascular endothelial dysfunction has been demonstrated in patients with migraine, which might explain the increased prevalence of RP in patients with migraine.

The avoidance of cold temperatures is the best method to prevent an episode of Raynaud's phenomenon. Keeping the whole body warm by wearing loose-fitting clothing, stockings, headwear and gloves in cold weather is a key strategy.7 A variety of factors can potentially aggravate the disorder and should be avoided, including smoking, caffeine, the use of sympathomimetic drugs and nonselective beta-blockers. Traditional pharmacological drugs alleviate RP symptoms by reducing vasoconstriction, inducing vasodilatory effect such as topical nitrate, calcium-channel blockers.

In this study, we aim to elucidate the clinical and laboratory characteristics that will facilitate the identification of PRP as well as the concomitant diseases.

Section snippets

Material and methods

A total of 58 cases followed up at the department of Pediatric Immunology and Rheumatology, Uludag University Faculty of Medicine between January 2007 and December 2015 were enrolled in our study. International consensus criteria for the diagnosis of Raynaud's Phenomenon were used to diagnose patients with RP in our study.8 A characteristic “triphasic” color pattern, (pallor, cyanosis, rubor) as well as numbness and swelling were the presenting symptoms of the patients. Patients older than 18

Results

We evaluated 20 (34.5%) male and 38 (65.5%) female patients with PRP in this study. Their ages ranged between 6 and 21 years; the mean age of the patients at diagnosis was 15.74 ± 2.88 years. Biphasic change in the skin color of the digits was observed in 65.5% (n = 38) whereas in 34.5% (n = 20) of the patients had triphasic color change. While being exposed to cold took first place in the investigation of the triggering factors, stress and exercise were in the following order (72.4% and 29.3%),

Discussion

Primary RP is more common in children than secondary RP. The onset of the symptoms of PRP occurs during adolescence period. Considering the limited number of studies made on PRP in children, we found out that girls are more predisposed to develop PRP and the onset occurs during the changes observed around menarche, due to the influence of sex hormones. Similarly, our study revealed that the mean age of symptom onset was around adolescence. The largest cohort published in 2003, involving 123

Funding

The authors declare that they have no source of funding for the performance of this study.

Conflict of interests

The authors declare they have no conflicts of interest.

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