Some remarks on management of juvenile chronic arthritis complicated by amyloidosis.

Acta Universitatis Carolinae. Medica Pub Date : 1994-01-01
K Rostropowicz-Denisiewicz
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Abstract

A retrospective study of 66 juvenile arthritic patients with reactive amyloidosis for a mean 12 +/- 10.7 years (1 to 30 years) from the onset of amyloidosis was performed. Forty-seven of them received continuous long-term chlorambucil therapy. Nineteen remaining patients were not treated with cytostatics of less than 3 months. The mortality rate for the whole group was 54.5%. 63.8% of those treated with chlorambucil are alive (mean survival 23.1 +/- 2.5 years). All patients from untreated group died. Their mean survival was 3.2 +/- 2.2 years. Nine patients from another group had been given thymus peptides for six months. Recurrent infections of the urinary and respiratory tract was the main reason for this therapy. Follow-up of 1 to 6 years showed good therapeutic effect of thymus peptides on the disease activity, alleviation of the renal manifestations and decline in recurrent infections. Recently, immunotherapy with intravenous immunoglobulins has been applied to 13 patients with IgG deficiency, renal failure, and persistent nephrotic syndrome. Preliminary results of the immunoglobulin treatment encourage to the further study on this therapeutic method.

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青少年慢性关节炎并发淀粉样变的治疗刍议。
回顾性研究66例反应性淀粉样变性青少年关节炎患者,平均12±10.7年(1 ~ 30年)从淀粉样变性发病。其中47例接受持续长期氯苯丁酸治疗。其余19例患者未使用细胞抑制剂治疗少于3个月。全组死亡率为54.5%。63.8%的使用氯霉素治疗的患者存活(平均生存23.1±2.5年)。未治疗组患者全部死亡。他们的平均生存期为3.2±2.2年。另一组的9名患者服用了6个月的胸腺肽。泌尿和呼吸道的反复感染是这种治疗的主要原因。随访1 ~ 6年,胸腺肽对疾病活动性、肾脏表现减轻、复发感染减少有良好的治疗效果。近期,对13例IgG缺乏、肾功能衰竭、持续性肾病综合征患者应用静脉注射免疫球蛋白免疫治疗。免疫球蛋白治疗的初步结果鼓励了这种治疗方法的进一步研究。
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