Akikusa J D, Schneider R, Harvey E A, Hebert D, Thorner P S, Laxer R M, Silverman E D
Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Arthritis Rheum. 2007 Jun 15;57(5):837-44. doi: 10.1002/art.22774.
Wegener's granulomatosis (WG) is a predominantly small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). There are few reports describing its clinical features and outcome in children. We report on the experience at a single tertiary referral center over 21 years.
We conducted a retrospective chart review of all patients diagnosed with WG at The Hospital for Sick Children between 1984 and 2005.
Twenty-five patients were identified. Median age at diagnosis and median followup were 14.5 years and 32.7 months, respectively. Male-to-female ratio was 1:4. Median duration of symptoms before diagnosis was 2 months. Of 22 patients, 21 were ANCA positive during their disease course (classic ANCA 78.9%). Constitutional symptoms were the most common clinical feature at presentation (24 of 25). Glomerulonephritis was present in 22 patients at presentation. Only 1 of 11 patients who presented with or developed renal impairment had normalization of serum creatinine. Upper airway involvement occurred in 21 patients at presentation and 24 over followup; only 1 had subglottic stenosis. Twenty patients had initial pulmonary involvement, most commonly nodules (44%) and pulmonary hemorrhage (44%). Five patients required ventilation for pulmonary hemorrhage. Four patients (16%) had venous thrombotic events (VTEs). Treatment included prednisone (100%), cyclophosphamide (76%), azathioprine (40%), and methotrexate (32%).
Pediatric WG typically presents in adolescence and has a female predominance. Glomerulonephritis and pulmonary disease are common at diagnosis and frequently present as a pulmonary-renal syndrome. Loss of renal function is common and rarely completely reversible. As in adults, children with WG are at risk of VTEs.
韦格纳肉芽肿（WG）是一种主要与抗中性粒细胞胞浆抗体（ANCA）相关的小血管炎。关于其在儿童中的临床特征和预后的报道较少。我们报告一家三级转诊中心21年来的经验。
我们对1984年至2005年期间在病童医院被诊断为WG的所有患者进行了回顾性病历审查。
共确定了25例患者。诊断时的中位年龄和中位随访时间分别为14.5岁和32.7个月。男女比例为1:4。诊断前症状的中位持续时间为2个月。在22例患者中，21例在病程中ANCA呈阳性（典型ANCA占78.9%）。全身症状是最常见的临床表现（25例中有24例）。22例患者就诊时存在肾小球肾炎。11例出现或发展为肾功能损害的患者中只有1例血清肌酐恢复正常。21例患者就诊时出现上呼吸道受累，随访期间有24例；只有1例有声门下狭窄。20例患者最初有肺部受累，最常见的是结节（44%）和肺出血（44%）。5例患者因肺出血需要通气。4例患者（16%）发生静脉血栓事件（VTE）。治疗包括泼尼松（100%）、环磷酰胺（76%）、硫唑嘌呤（40%）和甲氨蝶呤（32%）。
儿童WG通常在青春期发病，女性居多。肾小球肾炎和肺部疾病在诊断时很常见，常表现为肺肾综合征。肾功能丧失很常见，很少完全可逆。与成人一样，WG患儿有发生VTE的风险。
