Original ArticleRetrospective view of primary Raynaud's phenomenon in childhoodRetrospectiva del fenómeno de Raynaud primario en la infancia
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.
References (29)
- et al.
Prevalence of Raynaud phenomenon in the adult population of South Carolina
J Clin Epidemiol
(1990) - et al.
Prevalence of Raynaud phenomenon in the general population. A preliminary study by questionnaire
J Chronic Dis
(1986) - et al.
International consensus criteria for the diagnosis of Raynaud's phenomenon
J Autoimmun
(2014) - et al.
Occurance of Raynaud's phenomenon in children ages 12–15 years: prevalence and association with other common symptoms
Artritis Rheum
(2003) - et al.
Geographic variation in the prevalence of Raynaud's phenomenon: Charleston, SC, USA, vs Tarentaise, Savoie, France
J Rheumatol
(1993) - et al.
Prevalence of Raynaud's phenomenon in patients with migraine
Arch Intern Med
(1984) - et al.
Association between Raynaud's phenomenon and migraine in a random population of hospital employees
J Rheumatol
(1993) Raynaud's phenomenon
N Engl J Med
(2002)The International classification of headache disorders: 2nd edition
Cephalalgia
(2004)- et al.
Raynaud's phenomenon in children: a retrospective review of 123 patients
Pediatrics
(2003)