National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Descartes, Paris, France.
Hôpital Necker-Enfants Malades, APHP, Institut des Maladies Génétiques (IMAGINE), Université Paris-Descartes, Paris, France.
Autoimmun Rev. 2018 Oct;17(10):984-989. doi: 10.1016/j.autrev.2018.08.001. Epub 2018 Aug 14.
To investigate differences between childhood (cPAN)- and adult-onset polyarteritis nodosa (aPAN) patients.
cPAN patients' clinical findings at onset and outcomes were compared to those of aPAN patients from the French Vasculitis Study Group registry matched for year of enrollment and initial systemic versus cutaneous disease. Their information on medications, disease activity and damage were collected. Kaplan-Meier relapse-free survival curves and the log-rank test were used to analyze cPAN versus aPAN differences for predefined outcomes.
Twenty-one children with systemic and 13 with cutaneous PAN were compared with 84 systemic- and 27 cutaneous-matched aPAN patients. Median follow-up exceeded 5 years for both groups. At study entry, mononeuritis multiplex was less frequent in systemic cPAN than systemic aPAN (P = 0.04), and purpura and myalgias were less frequent in cutaneous cPAN than cutaneous aPAN (P < 0.03). During follow-up, systemic cPAN relapsed more often than matched systemic aPAN (P < 0.0001), while relapse rates were similar for cutaneous disease (P > 0.05). Mostly minor relapses, predominantly involving the skin, occurred in all 4 groups. At last visit, damage accrual was comparable for cPAN and aPAN patients, but fewer systemic cPAN patients were treatment-free (15% versus 42%; P = 0.03). Two (6%) cPAN and 8 (7%) aPAN patients died.
Systemic PAN is equally severe in children and adults and carries a higher risk of relapse. The main cutaneous PAN features seem not to be influenced by age at disease onset.
探讨儿童起病型（cPAN）和成人起病型多动脉炎（aPAN）患者之间的差异。
将 cPAN 患者发病时的临床发现和结局与法国血管炎研究组登记处的 aPAN 患者进行比较，这些患者按入组年份和初始系统性与皮肤性疾病进行匹配。收集了他们关于药物、疾病活动和损害的数据。采用 Kaplan-Meier 无复发生存曲线和对数秩检验分析 cPAN 与 aPAN 在预设结局方面的差异。
将 21 例系统性和 13 例皮肤性 PAN 患儿与 84 例系统性和 27 例皮肤性匹配的 aPAN 患者进行比较。两组的中位随访时间均超过 5 年。在研究入组时，多发性单神经炎在系统性 cPAN 中比系统性 aPAN 中少见（P=0.04），而在皮肤性 cPAN 中，紫癜和肌痛比皮肤性 aPAN 中少见（P<0.03）。在随访期间，系统性 cPAN 的复发率高于匹配的系统性 aPAN（P<0.0001），而皮肤性疾病的复发率相似（P>0.05）。所有 4 组均发生了主要为轻微的、主要累及皮肤的复发。在最后一次就诊时，cPAN 和 aPAN 患者的累积损害相当，但更少的系统性 cPAN 患者无需治疗（15%比 42%；P=0.03）。2 例（6%）cPAN 和 8 例（7%）aPAN 患者死亡。
儿童系统性 PAN 与成人一样严重，且复发风险更高。主要的皮肤性 PAN 特征似乎不受发病年龄的影响。
