Department of Pediatrics, National Taiwan University Hospital, No.8, Chung Shan South Road, Taipei, 10002, Taiwan.
Department of Medical Research, National Taiwan University Hospital, Taipei, Taiwan.
Pediatr Rheumatol Online J. 2020 Nov 10;18(1):86. doi: 10.1186/s12969-020-00480-3.
Though outcome differences between children and adults with immunoglobulin A vasculitis (IgAV) has been well documented, it remains unclear if disease features in pediatric IgAV patients vary with onset age. We aimed to explore clinical features and prognosis of pediatric IgAV stratified by onset age.
We retrospectively reviewed records of patients under 18 years old diagnosed with IgAV from January 1999 to December 2018 in one tertiary medical center in Taiwan. Patients were grouped by onset age: ≤ 6 years old, 6-12 years old (> 6, ≤ 12), and 12-18 years old (> 12, < 18). Demographics, laboratory data, incidence of gastrointestinal, renal, and joint involvement, corticosteroid dependence, recurrence, and refractory disease were analyzed. Recurrence was defined as disease flare-up after complete remission and discontinuation of all medications for at least 3 months. Corticosteroid dependence was defined by more than 6 weeks of daily oral corticosteroid intake. Refractory disease was defined as not achieving complete remission 6 months after disease onset. Statistical analysis was performed using R software (v3.6.0).
There were 484 IgAV patients, with an onset age of 6.10 (4.72-8.58) (median (IQR)) years old. There were 234 (48.3%) patients ≤6 years old, 210 (43.4%) 6-12 years old, and 40 (8.3%) 12-18 years old. One hundred and thirty (26.9%) patients had renal involvement, which was more frequent in older children (≤ 6 years old, 18.4%; 6-12 years old, 31.0%; 12-18 years old, 55.0%; p < 0.001). There were 361 patients (74.6%) with joint involvement; younger children were affected more frequently (≤ 6 years old, 82.1%; 6-12 years old, 71.9%; 12-18 years old, 45.0%; p < 0.001). Gastrointestinal involvement was present in 311 (64.3%) patients, showing no difference among age groups. There were 46 patients (9.5%) with recurrent IgA vasculitis, 136 (28.1%) with corticosteroid dependent and 76 (15.7%) with refractory disease. Corticosteroid dependence and refractory disease occurred more frequently as onset age increased (p < 0.001).
Pediatric IgAV with different onset ages are associated with distinct clinical manifestations and outcomes. The risk of developing corticosteroid dependence, refractory disease and renal involvement increased with onset age.
虽然儿童和成人免疫球蛋白 A 血管炎（IgAV）之间的结局差异已有充分的文献记载，但儿童 IgAV 患者的疾病特征是否随发病年龄而变化仍不清楚。我们旨在探讨按发病年龄分层的儿科 IgAV 的临床特征和预后。
我们回顾性分析了 1999 年 1 月至 2018 年 12 月期间在台湾一家三级医疗中心诊断为 IgAV 的 18 岁以下患者的记录。患者按发病年龄分组：≤6 岁，6-12 岁（＞6，≤12）和 12-18 岁（＞12，＜18）。分析了人口统计学、实验室数据、胃肠道、肾脏和关节受累的发生率、皮质类固醇依赖性、复发和难治性疾病。复发定义为完全缓解后且所有药物停药至少 3 个月后疾病复发。皮质类固醇依赖性定义为需要每天口服皮质类固醇 6 周以上。难治性疾病定义为疾病发病后 6 个月未达到完全缓解。使用 R 软件（v3.6.0）进行统计分析。
共纳入 484 例 IgAV 患者，发病年龄为 6.10（4.72-8.58）（中位数（IQR））岁。234 例（48.3%）患者≤6 岁，210 例（43.4%）为 6-12 岁，40 例（8.3%）为 12-18 岁。130 例（26.9%）患者存在肾脏受累，年龄较大的儿童更常见（≤6 岁，18.4%；6-12 岁，31.0%；12-18 岁，55.0%；p<0.001）。361 例（74.6%）患者存在关节受累；年龄较小的儿童更常见（≤6 岁，82.1%；6-12 岁，71.9%；12-18 岁，45.0%；p<0.001）。311 例（64.3%）患者存在胃肠道受累，各年龄组间无差异。46 例（9.5%）患者出现复发性 IgA 血管炎，136 例（28.1%）患者需要皮质类固醇依赖，76 例（15.7%）患者为难治性疾病。皮质类固醇依赖性和难治性疾病的发生率随发病年龄的增加而增加（p<0.001）。
不同发病年龄的儿科 IgAV 与不同的临床表现和结局相关。皮质类固醇依赖、难治性疾病和肾脏受累的风险随发病年龄的增加而增加。
