<p>Raynaud phenomenon (RP) is characterized by recurrent discoloration of the fingers or toes due to vasospasm of peripheral blood vessels. Diagnosis is based on clinical evaluation and physical examination. The prevalence of RP in the pediatric population is 18% in girls and 12% in boys. Among children in their first decade of life, the prevalence is approximately 2%. There are two forms of RP: primary and secondary. Primary RP shows no significant pathological findings on nailfold capillaroscopy (NFC), and antinuclear antibody (ANA) levels typically remain below 1:640. In contrast, secondary RP is associated with fewer than six capillaries per millimeter and an increased number of abnormal capillaries, such as giant capillaries, hemorrhages, and avascular areas. It is crucial to define what constitutes normal nailfold capillaroscopy findings in children, as they differ from those in adults. A&#xa0;recently published systematic review demonstrated that 70% of 215 patients exhibited RP at the onset of disease. In the world’s largest juvenile systemic sclerosis cohort (<a href="http://www.juvenile-scleroderma.com">www.juvenile-scleroderma.com</a>), 90% of 150 patients had RP after an average disease duration of 2.4&#xa0;years. Regarding follow-up, if NFC findings are pathological, the patient should be reassessed every 6&#xa0;months. If pathological NFC findings are accompanied by specific ENA (extractable nuclear antigen) positivity, follow-up should occur every 3&#xa0;months.</p>

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Raynaud-Phänomen bei Kindern und Jugendlichen

  • Ivan Foeldvari

摘要

Raynaud phenomenon (RP) is characterized by recurrent discoloration of the fingers or toes due to vasospasm of peripheral blood vessels. Diagnosis is based on clinical evaluation and physical examination. The prevalence of RP in the pediatric population is 18% in girls and 12% in boys. Among children in their first decade of life, the prevalence is approximately 2%. There are two forms of RP: primary and secondary. Primary RP shows no significant pathological findings on nailfold capillaroscopy (NFC), and antinuclear antibody (ANA) levels typically remain below 1:640. In contrast, secondary RP is associated with fewer than six capillaries per millimeter and an increased number of abnormal capillaries, such as giant capillaries, hemorrhages, and avascular areas. It is crucial to define what constitutes normal nailfold capillaroscopy findings in children, as they differ from those in adults. A recently published systematic review demonstrated that 70% of 215 patients exhibited RP at the onset of disease. In the world’s largest juvenile systemic sclerosis cohort (www.juvenile-scleroderma.com), 90% of 150 patients had RP after an average disease duration of 2.4 years. Regarding follow-up, if NFC findings are pathological, the patient should be reassessed every 6 months. If pathological NFC findings are accompanied by specific ENA (extractable nuclear antigen) positivity, follow-up should occur every 3 months.