Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo 157-8535, Japan.
Pediatr Nephrol. 2012 Apr;27(4):681-5. doi: 10.1007/s00467-011-2085-5. Epub 2011 Dec 31.
Antiphospholipid syndrome (APS) is a rare disorder in children. More than half of childhood APS occurs as secondary APS complicated by systemic lupus erythematosus (SLE) and other autoimmune diseases.
CASE-DIAGNOSIS/TREATMENT: We encountered a boy with SLE who presented with thrombotic microangiopathy (TMA) due to APS. He was initially diagnosed with SLE and treated with methylprednisolone pulse therapy. However, his renal function rapidly deteriorated. Since poikilocytes were detected, we suspected that his condition was complicated by TMA or APS. Urgent plasma exchange, continuous hemodialysis, and intravenous cyclophosphamide saved the patient and his renal failure ameliorated. A renal biopsy performed at the onset of disease showed multiple microvascular thrombi, diffuse mesangiolysis, and cortical necrosis compatible with TMA. He was positive for anticardiolipin antibody, anti-β2-glycoprotein I antibody, and lupus anticoagulant as well as anti-phosphatidylserine-prothrombin complex IgG antibody (aPS/PT). Anti-a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) antibody was negative and ADAMTS13 activity was normal. The aPS/PT is thrombogenic and is a newly discovered lupus anticoagulant.
Childhood TMA due to APS has rarely been reported. To the best of our knowledge this is the first report of pediatric TMA due to APS with positive aPS/PT. Physicians need to be aware of aPS/PT in pediatric APS and/or SLE.
抗磷脂综合征（APS）在儿童中较为罕见。超过一半的儿童 APS 为继发性 APS，与系统性红斑狼疮（SLE）和其他自身免疫性疾病有关。
病例诊断/治疗：我们遇到一位患有 SLE 的男孩，其因 APS 而出现血栓性微血管病（TMA）。他最初被诊断为 SLE，并接受甲基强的松龙脉冲治疗。然而，他的肾功能迅速恶化。由于发现了异形红细胞，我们怀疑他的病情并发 TMA 或 APS。紧急血浆置换、持续血液透析和静脉注射环磷酰胺挽救了患者，他的肾衰竭得到改善。疾病发作时进行的肾活检显示多发性微血栓、弥漫性系膜溶解和皮质坏死，符合 TMA。他的抗心磷脂抗体、抗β2-糖蛋白 I 抗体和狼疮抗凝物以及抗磷脂酰丝氨酸-凝血酶原复合物 IgG 抗体（aPS/PT）呈阳性。抗去整合素样金属蛋白酶与凝血酶 13 型（ADAMTS13）抗体阴性，ADAMTS13 活性正常。aPS/PT 具有促血栓形成作用，是一种新发现的狼疮抗凝物。
儿童 APS 引起的 TMA 很少见。据我们所知，这是首例儿童 APS 伴阳性 aPS/PT 引起的 TMA 报告。儿科医生需要注意儿科 APS 和/或 SLE 中的 aPS/PT。
