Regional Nephrology Unit, Belfast City Hospital, Belfast, Northern Ireland.
Am J Transplant. 2012 Apr;12(4):1046-51. doi: 10.1111/j.1600-6143.2011.03923.x. Epub 2012 Jan 10.
Dense deposit disease is a rare glomerulonephritis caused by uncontrolled stimulation of the alternative complement pathway. Allograft survival after kidney transplantation is significantly reduced by the high rate of disease recurrence. No therapeutic interventions have consistently improved outcomes for patients with primary or recurrent disease. This is the first reported case of recurrent dense deposit disease being managed with eculizumab. Within 4 weeks of renal transplantation, deteriorating graft function and increasing proteinuria were evident. A transplant biopsy confirmed the diagnosis of recurrent dense deposit disease. Eculizumab was considered after the failure of corticosteroid, rituximab and plasmapheresis to attenuate the rate of decline in allograft function. There was a marked clinical and biochemical response following the administration of eculizumab. This case provides the first evidence that eculizumab may have a place in the management of crescentic dense deposit disease. More information is necessary to clarify the effectiveness and role of eculizumab in dense deposit disease but the response in this patient was encouraging. The results of clinical trials of eculizumab in this condition are eagerly awaited.
致密物沉积病是一种罕见的肾小球肾炎，由替代补体途径不受控制的刺激引起。移植肾的移植物存活率因疾病复发率高而显著降低。对于原发性或复发性疾病患者，没有治疗干预措施能始终改善其结局。这是首例使用依库珠单抗治疗复发性致密物沉积病的报道病例。肾移植后 4 周内，移植肾功能逐渐恶化，蛋白尿逐渐增加。移植肾活检证实了复发性致密物沉积病的诊断。在皮质类固醇、利妥昔单抗和血浆置换未能减轻移植物功能下降速度后，考虑使用依库珠单抗。依库珠单抗治疗后，患者的临床和生化反应明显。该病例首次提供了依库珠单抗可能在新月体致密物沉积病治疗中发挥作用的证据。需要更多信息来阐明依库珠单抗在致密物沉积病中的有效性和作用，但该患者的反应令人鼓舞。人们急切地等待着该条件下依库珠单抗临床试验的结果。
