Pavlov-Dolijanović Slavica, Damjanov Nemanja, Ostojić Predrag, Susić Gordana, Stojanović Roksanda, Gacić Dragica, Grdinić Aleksandra
Institute of Rheumatology-Belgrade, Resavska, Belgrade, Serbia and Montenegro.
Pediatr Dermatol. 2006 Sep-Oct;23(5):437-42. doi: 10.1111/j.1525-1470.2006.00278.x.
To assess the prognostic value of capillaroscopy findings for the development of connective tissue disease in children and adolescents with Raynaud phenomenon, we followed up a group of 250 (mean age 15 years) for 1 to 6 years after the first capillaroscopy was performed. Every 6 months they were screened for signs and symptoms of connective tissue disease. Analysis was performed on capillary changes registered 6 months before the development of connective tissue disease. Capillary changes were classified into three types: normal, nonspecific, and sclerodermatous. At the end of the follow-up period, 191 (76%) subjects had primary Raynaud phenomenon, 27 (10.8%) were diagnosed as having undifferentiated connective tissue disease, and 32 (12.8%) fulfilled the criteria for a diagnosis of a specific connective tissue disease. Systemic lupus erythematosus was found in nine (3.6%) patients, rheumatoid arthritis in 10 (4%) patients (six of them with juvenile onset rheumatoid arthritis), and scleroderma spectrum disorders in 13 (5.2%). The mean time for the evolution of Raynaud phenomenon into undifferentiated connective tissue disease or a form of the disease was 2 years. Most of the subjects with primary Raynaud phenomenon (173/191, 91%), undifferentiated connective tissue disease (22/27, 81%), juvenile onset rheumatoid arthritis/rheumatoid arthritis (7/10, 70%), and systemic lupus erythematosus (6/9, 67%) had normal capillary findings. Nonspecific capillary changes occurred in 3 of 10 (30%) patients with rheumatoid arthritis, 2 of 9 (22%) with systemic lupus erythematosus, 4 of 27 (15%) with undifferentiated connective tissue disease, and 18 of 191 (9%) with primary Raynaud phenomenon. Of all the subjects, only 10 (4%) showed sclerodermatous disease type capillary changes 6 months before the expression of a particular disease: eight (62%) of these developed scleroderma spectrum disorders, one expressed systemic lupus erythematosus, and one had undifferentiated connective tissue disease. We concluded that there were no specific capillary changes predictive for future development of systemic lupus erythematosus, juvenile onset rheumatoid arthritis/rheumatoid arthritis, and undifferentiated connective tissue disease in children and adolescents with Raynaud phenomenon. Most of our study subjects with Raynaud phenomenon who developed these diseases had normal capillary findings or nonspecific changes. Children and adolescents who developed scleroderma spectrum disorders showed a sclerodermatous type of capillary changes 6 months before the expression of the disease, indicating that this type of capillary changes in children and adolescents with Raynaud phenomenon highly correlated with further development of scleroderma spectrum disorders.
为评估毛细血管镜检查结果对患有雷诺现象的儿童和青少年发生结缔组织病的预后价值，我们对一组250名（平均年龄15岁）患者在首次进行毛细血管镜检查后进行了1至6年的随访。每6个月对他们进行结缔组织病体征和症状筛查。对结缔组织病发生前6个月记录的毛细血管变化进行分析。毛细血管变化分为三种类型：正常、非特异性和硬皮病样。随访期结束时，191名（76%）受试者患有原发性雷诺现象，27名（10.8%）被诊断为未分化结缔组织病，32名（12.8%）符合特定结缔组织病的诊断标准。9名（3.6%）患者患有系统性红斑狼疮，10名（4%）患者患有类风湿关节炎（其中6名患有幼年型类风湿关节炎），13名（5.2%）患有硬皮病谱系障碍。雷诺现象演变为未分化结缔组织病或某种疾病形式的平均时间为2年。大多数原发性雷诺现象患者（173/191，91%）、未分化结缔组织病患者（22/27，81%）、幼年型类风湿关节炎/类风湿关节炎患者（7/10，70%）和系统性红斑狼疮患者（6/9，67%）的毛细血管检查结果正常。10名类风湿关节炎患者中有3名（30%）、9名系统性红斑狼疮患者中有2名（22%）、27名未分化结缔组织病患者中有4名（15%）以及191名原发性雷诺现象患者中有18名（9%）出现非特异性毛细血管变化。在所有受试者中，只有10名（4%）在某种特定疾病表现前6个月出现硬皮病样毛细血管变化：其中8名（62%）发展为硬皮病谱系障碍，1名表现为系统性红斑狼疮，1名患有未分化结缔组织病。我们得出结论，对于患有雷诺现象的儿童和青少年，没有特定的毛细血管变化可预测系统性红斑狼疮、幼年型类风湿关节炎/类风湿关节炎和未分化结缔组织病的未来发展。我们研究中大多数出现这些疾病的雷诺现象受试者的毛细血管检查结果正常或有非特异性变化。出现硬皮病谱系障碍的儿童和青少年在疾病表现前6个月显示出硬皮病样毛细血管变化，这表明患有雷诺现象的儿童和青少年的这种类型的毛细血管变化与硬皮病谱系障碍的进一步发展高度相关。
