OBJECTIVE:Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. We aimed to examine the characteristics of adult- and childhood-onset PAN. METHODS:Fifteen pediatric (˂ 18 years) and 22 adult PAN patients who fulfilled the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively, were included in the study. RESULTS:Five children had cutaneous and all the rest of the patients had systemic PAN. Weight loss was more common (59.1% vs. 20%, P = 0.041) and presence of an angiography at diagnosis was more frequent (81.8% vs. 33.3%, P = 0.003) in adults than children. Arthralgia/arthritis and skin involvement were more common in children (86.7% vs. 59.1%; 93.3% vs. 72.7%, respectively) while renal and neurologic involvement were more frequently observed in adult patients (50% vs. 20%; 59.1% vs. 40%, respectively) (P > 0.05 for all). Cutaneous PAN patients were treated with corticosteroids only. All but one adult patient received cyclophosphamide while mycophenolate mofetil was used in five and cyclophosphamide was used in four children as induction treatment. The median duration of induction treatment was longer in adults than children (12 vs. 3 months, respectively; P = 0.004). The most common maintenance drug was mycophenolate mofetil in children and azathioprine in adults. The mortality rate was 13.6% (n = 3) and 0% in adults and children, respectively. CONCLUSION:To our knowledge, this is the first study comparing characteristics of adult and childhood onset PAN. Our results have suggested that juvenile PAN had a more benign course (with less renal and neurologic involvement, shorter duration of induction treatment) than adult onset PAN.

译文

目的:结节性多发性动脉炎(PAN)是一种中/小动脉的坏死性血管炎。我们旨在检查成人和儿童期PAN的特征。
方法:本研究纳入了分别符合Ankara 2008年和1990年美国风湿病学会标准的15例儿科(˂18岁)和22例成年PAN患者。
结果:5名儿童皮肤皮肤,其余患者均患有全身性PAN。与儿童相比,成人的体重减轻更为普遍(59.1%vs. 20%,P = 0.041),并且在诊断时出现血管造影的频率更高(81.8%vs. 33.3%,P = 0.003)。儿童的关节痛/关节炎和皮肤受累更为常见(分别为86.7%和59.1%; 93.3%和72.7%),而成年患者更经常观察到肾脏和神经系统受累(50%和20%; 59.1%)分别为40%)(所有P均> 0.05)。皮肤PAN患者仅接受皮质类固醇激素治疗。除一名成年患者外,其余所有患者均接受环磷酰胺治疗,其中5例使用了麦考酚酸酯,四例儿童使用了环磷酰胺作为诱导治疗。成人的中位诱导治疗时间比儿童长(分别为12个月和3个月; P = 0.004)。最常见的维持性药物是儿童用霉酚酸酯和成人用硫唑嘌呤。成人和儿童的死亡率分别为13.6%(n = 3)和0%。
结论:据我们所知,这是第一项比较成人和儿童发病PAN的特征的研究。我们的结果表明,与成人发作的PAN相比,未成年人的PAN具有更好的病程(肾脏和神经系统受累较少,诱导治疗时间更短)。

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