Nephrogenic Systemic Fibrosis A Pathologic Study of Autopsy Cases
Nephrogenic Systemic Fibrosis A Pathologic Study of Autopsy Cases
复制标题
DOI：
10.1043/1543-2165-133.12.1943
复制
发表时间：
2009-12-01
影响因子：
4.6
通讯作者：
Kay, Jonathan
中科院分区：
文献类型：
--
作者：
Koreishi, Aashiyana F.;Nazarian, Rosalynn M.;Kay, Jonathan
背景。-肾源性系统性纤维化（NSF）是一种罕见但严重的疾病，最初被描述为一个纯粹的皮肤病过程。孤立的尸检报告描述了这种疾病的多器官受累。通过描述马萨诸塞州总医院的尸检经验，进一步说明NSF的各种系统性参与。我们描述的结果在一系列的4例尸检病例诊断为NSF。本报告描述了肾功能不全的历史，暴露于含钆造影剂，具体的实验室参数，并通过尸检确定全身受累的程度。死亡原因包括全身血栓栓塞性疾病（n = 3）和肺炎（n = 1）。实验室参数和含钆造影剂暴露的类型、剂量或时间与临床结果和结局无关。所有患者的皮肤表现的疾病和肾钙质沉着症，与一些表现出钙化和纤维化的硬脑膜，甲状腺，心脏，包括心脏传导系统，尸检。软组织钙化与并发甲状旁腺功能亢进或血清甲状旁腺激素水平升高相关。血栓栓塞性疾病可能是NSF的重要临床并发症。NSF患者还可能出现多器官纤维化和钙化的组织学特征，具有显著的发病率和死亡率。该尸检系列强调了NSF全身表现的变异性。(Arch Pathol Lab Med. 2009; 133：1943-1948）
Context.-Nephrogenic systemic fibrosis (NSF) is a rare but serious disorder initially described as a purely dermatologic process. Isolated autopsy reports have described multiorgan involvement by this disease.Objective.-To further illustrate the varied and systemic involvement of NSF by describing the autopsy experience at the Massachusetts General Hospital.Design.-We describe the findings in a series of 4 autopsy cases of patients diagnosed with NSF. This report describes the history of renal dysfunction, exposure to gadolinium-containing contrast agents, specific laboratory parameters, and the extent of systemic involvement identified by postmortem examination.Results.-Causes of death included systemic thromboembolic disease (n = 3) and pneumonia (n = 1). Laboratory parameters and type, dose, or timing of gadolinium-containing contrast-agent exposure did not correlate with clinical findings and outcomes. All patients demonstrated cutaneous manifestations of the disease and nephrocalcinosis, with some exhibiting calcification and fibrosis of the dura, thyroid, and heart including the cardiac conduction system, on postmortem examination. Soft tissue calcification was associated with concurrent hyperparathyroidism or high serum parathyroid hormone levels.Conclusions.-Thromboembolic disease can be a significant clinical complication of NSF. Patients with NSF may also develop characteristic histologic features of fibrosis and calcification in multiple organs, with significant morbidity and mortality. This autopsy series highlights the variability of systemic manifestations of NSF. (Arch Pathol Lab Med. 2009; 133: 1943-1948)