Service of Immunology and Rheumatology, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina.
J Rheumatol. 2013 Mar;40(3):329-34. doi: 10.3899/jrheum.120386. Epub 2013 Jan 15.
Systemic juvenile idiopathic arthritis (SJIA) frequently leads to disability and damage. Predictive factors for a poor outcome include persistent systemic features and younger age at onset. We describe and analyze disease features in patients with early-onset (EO) SJIA (disease onset before age 18 mo) and compare them to patients with later-onset (LO) disease.
Clinical features at onset, activity measures (occurrence of macrophage activation syndrome, remission), and outcome measures for disability [Childhood Health Assessment Questionnaire (CHAQ) ≥ 0.5] and damage [radiographic joint destruction, Juvenile Arthritis Damage Index (JADI) score, growth retardation] observed during followup were analyzed retrospectively in patients with SJIA followed for ≥ 3 years since disease onset.
In total 132 patients were included. SJIA started at age ≤ 18 months in 19 (14%) patients and at a later age in 113 (86%) children. At onset, serositis (p < 0.01) and hepatomegaly (p < 0.05) were more frequent in EO patients, who also exhibited lower hemoglobin levels (p < 0.03) and higher platelet counts (p < 0.03) than patients with LO. Macrophage activation syndrome occurred in 20 patients (11 EO and 9 LO; p < 0.0001). Remission was achieved by 49 patients (37%; 4 EO and 45 LO). At last visit, destructive hip disease (p < 0.04), growth retardation (p < 0.01), radiographic damage (p < 0.02), and disability (p < 0.04) were more frequent in patients with EO disease, who had higher JADI scores (p < 0.003).
Patients with EO exhibited a more aggressive and destructive disease course than patients with LO SJIA.
全身性幼年特发性关节炎（SJIA）常导致残疾和损伤。预后不良的预测因素包括持续的全身表现和发病年龄较小。我们描述和分析了早发性（EO）SJIA（发病年龄在 18 个月之前）患者的疾病特征，并将其与晚发性（LO）疾病患者进行了比较。
回顾性分析了 132 例 SJIA 患者的临床特征（发病时、活动指标[巨噬细胞活化综合征的发生、缓解]）和随访期间的残疾[儿童健康评估问卷（CHAQ）≥0.5]和损伤[关节放射学破坏、幼年关节炎损伤指数（JADI）评分、生长迟缓]的指标。所有患者的 SJIA 发病时间均≥3 年。
共纳入 132 例患者。19 例（14%）患者 SJIA 发病年龄≤18 个月，113 例（86%）患儿发病年龄较晚。发病时，EO 患者出现浆膜炎（p<0.01）和肝肿大（p<0.05）的频率更高，且血红蛋白水平较低（p<0.03）、血小板计数较高（p<0.03）。20 例患者（11 例 EO 和 9 例 LO；p<0.0001）发生巨噬细胞活化综合征。49 例患者（37%；4 例 EO 和 45 例 LO）达到缓解。末次随访时，EO 患者破坏性髋关节疾病（p<0.04）、生长迟缓（p<0.01）、放射学损伤（p<0.02）和残疾（p<0.04）的频率更高，JADI 评分更高（p<0.003）。
EO 患者的疾病病程较 LO SJIA 患者更具侵袭性和破坏性。
