丙种球蛋白无反应型川崎病的发病机制及治疗进展

Pathogenesis and treatment of intravenous immunoglobulin - resistant Kawasaki disease

川崎病是一种儿童期常见病，是一种病因未明的系统性血管炎，是儿童时期后天性心脏病的主要病因。川崎病的病因尚不明确，疾病本身可能是易感人群因为一个共同的免疫介导所致血管炎通路的特征性表现。静脉用丙种球蛋白（IVIG）是目前大家公认的治疗川崎病的一线药物，但仍有10％～20％的患儿对IVIG无反应，虽然IVIG会被选择再次应用，但有效率较初次应用更低，因此，对某些川崎病患儿来说单用IVIG是无效的，即使重复应用也是如此。目前，甲泼尼龙用于治疗IVIG无反应型川崎病，但如何有效而安全应用，还未完全阐明，此外，肿瘤坏死因子-α拮抗剂英夫利西单抗、环孢素、血浆置换等能有效治疗IVIG无反应型川崎病。

Kawasaki disease（KD） is an acute, self- limited vasculitis of childhood and has become the lea- ding eause of acquired pediatrie heart disease. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune - mediated vascular inflammation in susceptible hosts. In- travenous immunoglobulin （IVIG） is now widely accepted as the first - line therapy for KD. However, approximately 10% -20% of patients are resistant to IVIG. Although, an additional administration of IVIG is often chosen for the treatment of KD patients resistant to the first administration of IVIG, its efficaey is rePorted to be lower than that of the first I^IG dose. Thus, it is elear that some KD patients can not be treated successfully by IVIG alone, even if it is used repetitively. Currently,methylprednisolone（MP） is used for the treatment of IVIG -resistant KD. However, the proper use of MP for maximum effect and safety has not yet been elueidated. Tumor necrosis factor - α blocker, infliximab effective in the control of inflammation in patients with resistant KD. Cyelosporin treatment is also a promising option for patients with refractory KD. Plasma exchange was a safe, effective prophylactic measure against coronary artery lesions in children with KD refractory to intravenous gamma globulin therapy.