Unidad de Reumatología pediátrica, Servicio de Pediatría, Hospital Universitario 12 de Octubre, Madrid, Spain.
Servicio de Pediatría, Complejo Hospitalario Universitario de Toledo, Toledo, Spain.
An Pediatr (Engl Ed). 2022 Sep;97(3):172-178. doi: 10.1016/j.anpede.2022.03.004. Epub 2022 Apr 19.
(1) To describe the prevalence of IgA deficiency (IgAD), uveitis, coeliac disease (CD) and thyroid disorders in a multicentric cohort of patients diagnosed with JIA and, (2) to evaluate whether patients with JIA and IgAD present other autoimmune diseases more frequently than patients with normal serum levels of IgA.
Retrospective chart review of a cohort of patients diagnosed with JIA followed at the paediatric rheumatology units of two hospitals in Madrid, Spain.
A total of 193 patients were included. Of them, 123 were females (64%). Median age at disease onset was 5.6 years (IQR 2.5-9.7) and the median time of follow-up was 5.1 years (IQR 2.2-8.1). The three most common ILAR categories were oligoarticular (53%), polyarticular RF negative (20%) and enthesitis related arthritis (10%). Serum IgA levels were available in 172/193 (89%); 25/172 (15%) had selective (<7mg/dl, n=8) or partial (7-69mg/dl, n=17) IgAD. All the patients had periodic eye exams. Eighteen children (9%) had anterior uveitis, 15/18 chronic and 3/18 acute. Serum anti transglutaminase IgA, or IgG in IgAD were obtained in 135/193 (70%). Four children (3%) were diagnosed with CD either by intestinal biopsy (n=3) or by the combination of characteristic clinical, serological and genetic features (n=1); two of them had IgAD (p=0.12; OR=6.4; 95% CI 0.9-47.6). Only 1/153 (0.7%) patient had hyperthyrotropinemia with positive anti-thyroid antibodies and required replacement therapy.
Patients with JIA frequently present autoimmune comorbidities. IgAD does not seem to increase their prevalence, with the possible exception of CD.
(1) 描述在诊断为 JIA 的多中心患者队列中，IgA 缺乏症（IgAD）、葡萄膜炎、乳糜泻（CD）和甲状腺疾病的患病率，(2) 评估 JIA 伴 IgAD 的患者是否比血清 IgA 水平正常的患者更常出现其他自身免疫性疾病。
回顾性分析西班牙马德里两家医院儿科风湿病科诊断为 JIA 的患者队列。
共纳入 193 例患者，其中 123 例为女性（64%）。疾病发病年龄中位数为 5.6 岁（IQR 2.5-9.7），中位随访时间为 5.1 年（IQR 2.2-8.1）。最常见的 ILAR 分类为寡关节型（53%）、多关节型 RF 阴性（20%）和附着点相关关节炎（10%）。172/193 例（89%）患者血清 IgA 水平可获得，25/172 例（15%）有选择性（<7mg/dl，n=8）或部分（7-69mg/dl，n=17）IgAD。所有患者均定期进行眼部检查，18 例儿童（9%）患有前葡萄膜炎，15/18 例为慢性，3/18 例为急性。135/193 例（70%）获得 IgAD 患者血清抗转谷氨酰胺酶 IgA 或 IgG。4 例（3%）儿童通过肠活检（n=3）或结合特征性临床、血清学和遗传特征（n=1）诊断为 CD；其中 2 例有 IgAD（p=0.12；OR=6.4；95%CI 0.9-47.6）。仅 1/153（0.7%）患者出现促甲状腺素升高和抗甲状腺抗体阳性，需要替代治疗。
JIA 患者常伴有自身免疫性合并症。IgAD 似乎不会增加其患病率，但 CD 除外。
