[Nailfold videocapillaroscopy - a useful tool for screening patients with juvenile idiopathic arthritis at the risk of development of premature atherosclerosis]. / Wideokapilaroskopia lozyska okolopaznokciowego - uzyteczne narzedzie w diagnostyce pacjentów z mlodzienczym idiopatycznym zapaleniem stawów zagrozonych rozwojem przedwczesnych zmian miazdzycowych.

Inflammation of the vascular wall with endothelial dysfunction and subsequent activation of inflammatory immune response play pivotal roles in the early development of the atherosclerotic process not only in adults with rheumatoid arthritis (RA), but also in children with juvenile idiopathic arthritis (JIA). This hypothesis was supported by our findings from autopsy examination, revealing atherosclerosis lesions in about 30% children with JIA. The established methods of assessing pre-clinical atherosclerosis include measurement of biochemical markers of endothelium impairment and ultrasonographic examination of vessels (FMD, IMT). The authors suggest that revealing structural and functional impairment of peripheral microvessels by means of static and dynamic videocapillaroscopy can give clinicians a chance to identify even younger patients with JIA/RA at high risk of atherosclerosis.

Evaluation of heart rhythm variability and arrhythmia in children with systemic and localized scleroderma.

OBJECTIVE: To evaluate possible disturbances in autonomic regulation and cardiac arrhythmias in children with localized and systemic scleroderma. METHODS: There were 40 children included in the study: 20 with systemic and 20 with localized scleroderma. The control group comprised 20 healthy children. RESULTS: In 24-hour Holter recording, the average rate of sinus rhythm was significantly higher in the groups with systemic and localized scleroderma than in the control group, but there was no significant difference between them. The variability of heart rhythm in both groups was significantly decreased. In the group with systemic scleroderma, single supraventricular ectopic beats were observed in 20% and runs were seen in 40% of patients. In the group with localized scleroderma, supraventricular single ectopic beats occurred in 35% of patients and runs in 45% of those studied. Ventricular arrhythmia occurred in 2 children with systemic scleroderma, but in 1 child, it was complex. CONCLUSION: The most frequent cardiac arrhythmias in both types of scleroderma in children were of supraventricular origin, whereas ventricular arrhythmias did not occur very often. There were no significant differences in autonomic disturbances manifesting as a higher heart rate and decreased heart rate variability between localized and systemic scleroderma.

Localized scleroderma in childhood is not just a skin disease.

OBJECTIVE: Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma. METHODS: Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement. RESULTS: Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflux. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased. CONCLUSION: Extracutaneous manifestations of juvenile localized scleroderma developed in almost one-fourth of the children in this study. These extracutaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. This subgroup of patients with juvenile localized scleroderma should be evaluated extensively, treated more aggressively, and monitored carefully.