Yoshida N, Takahashi K, Kurozumi H, Hyodo Y, Yokota S
Department of Infection and Immunology, Kanagawa Children's Medical Center, Yokohama-city.
Ryumachi. 1990 Apr;30(2):119-25.
A case of polyarteritis nodosa (PN) in childhood involving various organs such as the gastrointestinal tract, skin, CNS, kidneys and liver with hypogammaglobulinemia is reported. This 6 month old girl was admitted to our hospital with vomiting, diarrhea, bloody stools with mucous and weight loss. For the past 5 months she had these abdominal symptoms. She was diagnosed as having PN of the Kussmaul-Maier variety on the grounds of the biopsy of skin lesion where a necrotizing vasculitis was found. Prednisolone and methylprednisolone pulse treatment were not effective in suppressing the progress of the disease. At the age of 1 year 7 month a combination therapy of prednisolone and immunosuppressants (cyclophosphamide) was started and this was found to be effective. She was discharged when she was 2 year and 2 month. The dosage of prednisolone was tapered as the activity of the PN decreased and she did well with a maintainance dosage of 9.5 mg/day. At 3 year 6 month of age she suddenly developed hypertension (the plasma renin activity was found to be 16.6 ng/m/hr. and the aldosterone 220 ng/dl). CNS involvement such as spinal cord dysfunction, left sided convulsions, cerebral hemorrhage developed 5 months later. Methylprednisolone pulse therapy was performed 3 times and 2 mg/kg/day of prednisolone was administered. In spite of this therapy she passed away with a massive cerebral hemorrhage at the age of 4 year 8 month. Unfortunately an autopsy was not performed. Results of the immunological tests proved that the hypogammaglobulinemia was a common variable immunodeficiency (CVI). It has been reported that primary immuno-deficiency syndrome is often associated with collagen disease and auto-immune disease. This lack of the defense mechanism against the virus or extra antigen could be related to the onset of collagen and auto-immune disease. As the correlation between CVI and PN has not been clarified this case is of interest as concerns the cause of PN.
报告了一例儿童结节性多动脉炎（PN），累及胃肠道、皮肤、中枢神经系统、肾脏和肝脏等多个器官，并伴有低丙种球蛋白血症。这名6个月大的女孩因呕吐、腹泻、黏液血便和体重减轻入院。在过去的5个月里，她一直有这些腹部症状。根据皮肤病变活检发现坏死性血管炎，她被诊断为Kussmaul-Maier型PN。泼尼松龙和甲泼尼龙脉冲治疗未能有效抑制疾病进展。在1岁7个月时开始使用泼尼松龙和免疫抑制剂（环磷酰胺）联合治疗，发现有效。她在2岁2个月时出院。随着PN活动度降低，泼尼松龙剂量逐渐减少，她以9.5毫克/天的维持剂量情况良好。在3岁6个月时，她突然出现高血压（血浆肾素活性为16.6纳克/毫升/小时，醛固酮为220纳克/分升）。5个月后出现中枢神经系统受累，如脊髓功能障碍、左侧惊厥、脑出血。进行了3次甲泼尼龙脉冲治疗，并给予2毫克/千克/天的泼尼松龙。尽管进行了这种治疗，她还是在4岁8个月时因大量脑出血去世。遗憾的是未进行尸检。免疫测试结果证明低丙种球蛋白血症是一种常见变异型免疫缺陷（CVI）。据报道，原发性免疫缺陷综合征常与胶原病和自身免疫性疾病相关。这种针对病毒或外来抗原的防御机制缺乏可能与胶原病和自身免疫性疾病的发病有关。由于CVI与PN之间的相关性尚未阐明，该病例对于PN的病因具有研究意义。
