Circulatory System in Children with Localized Scleroderma

Abstract

The circulatory system was studied in 43 children (ages 3-18 years, mean 10.6 years; 32 girls, 11 boys) suffering from localized cutaneous forms of scleroderma. The following studies were undertaken: general pediatric examination, cardiologic examination including routine electrocardiogram (ECG), 24-h Holter ECG monitoring, echocardiography plus Doppler study, and a treadmill exercise test. Three children found to have congenital heart malformations were excluded from the study. Children with localized scleroderma were often lighter and shorter than their appropriate controls. The most common ECG abnormality was incomplete right bundle branch block, but other ECG and Holter abnormalities were found as well. Abnormal echocardiographic results were obtained in 16 cases. The abnormalities concerned valvar function (in all heart valves but predominantly in the mitral valve). It was found that the children suffering from scleroderma had different indices than controls for left ventricular mass index, mitral valve function and left ventricular filling. During the exercise test (conducted according to Bruce's protocol) we found no difference between patients and controls.

Systemic manifestations in localized scleroderma

“…Electrocardiogram abnormalities, mainly represented by right bundle branch block, have been reported in a group of 43 children with LS systematically evaluated. Abnormal valve function, particularly of mitral valve, was found in 16 patients (37%) while exercise test was normal in all [44]. The meaning of these findings has not been fully explained by the authors.…”

Localized scleroderma: clinical and epidemiological features with emphasis on adulthood‐ versus childhood‐onset disease differences

“…Gastroesophageal reflux, dysphagia, restrictive lung disease, abdominal pain and Raynaud phenomenon (RP) were observed in 0.7–7% of children and were most common in the generalized JLS . A pilot studies on a small JLS cohorts revealed circulatory system and oesophageal abnormalities, but no further findings in a larger cohorts were published . Similar manifestations were also described in adults.…”

Localized scleroderma in childhood is not just a skin disease

“…However, although pericarditis has never been described in children with juvenile localized scleroderma, electrocardiographic abnormalities, mainly represented by incomplete right bundle branch block, have been reported in both children and in adults (35,36).…”