Schieren Gisela, Wirtz Nikolaus, Altmeyer Peter, Rump Lars Christian, Weiner Stefan Markus, Kreuter Alexander
Department of Nephrology, University Hospital, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.
J Am Acad Dermatol. 2009 Nov;61(5):868-74. doi: 10.1016/j.jaad.2009.03.040.
Nephrogenic systemic fibrosis (NSF) affects some patients on dialysis after gadolinium contrast agent-enhanced magnetic resonance imaging. It is characterized by progressive skin fibrosis of the extremities, sometimes including the trunk and internal organs.
The clinical course of 10 patients with biopsy-proven NSF was analyzed retrospectively with regard to gadolinium exposition, disease onset, and progression of NSF with special emphasis on physical mobility and impact of different therapeutic approaches.
Despite physiotherapy and different additional therapeutic approaches (eg, immunosuppression, ultraviolet A-1 phototherapy, or extracorporal photopheresis) all patients developed progressive skin fibrosis of the lower extremities, sometimes including the trunk and arms. Kidney transplantation led to a slow improvement of skin lesions in one patient. Nine patients developed progressive joint contractures, and 8 patients became wheelchair bound within 12 months after disease onset and became dependent on the support of family members or a nursing service.
Retrospective analysis in a relatively small number of patients is a limitation.
NSF appears to be a rapidly progressive disabling disease with limited therapeutic options.
钆对比剂增强磁共振成像后，肾源性系统性纤维化（NSF）会影响一些透析患者。其特征为四肢进行性皮肤纤维化，有时累及躯干和内脏。
回顾性分析10例经活检证实为NSF患者的临床病程，涉及钆暴露情况、疾病起病及NSF的进展，特别关注身体活动能力以及不同治疗方法的影响。
尽管进行了物理治疗及不同的其他治疗方法（如免疫抑制、紫外线A-1光疗或体外光分离置换法），所有患者均出现下肢进行性皮肤纤维化，有时累及躯干和手臂。1例患者肾移植后皮肤病变缓慢改善。9例患者出现进行性关节挛缩，8例患者在疾病起病后12个月内需要依赖轮椅，并需家人或护理服务的支持。
对相对少数患者进行回顾性分析是一个局限。
NSF似乎是一种进展迅速的致残性疾病，治疗选择有限。
