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肾源性系统性硬化症(nephrogenic systemic fibrosis,NSF)是发生在肾功能不全患者因注射MRI造影剂而引发的系统性硬化症,累及全身多个器官,但以下肢为首发。初发症状为皮肤红斑、僵硬,病情进展累及全身多个器官,最终因器官衰竭而死亡。皮肤活检示真皮层内大量胶原和纤维组织增生,确诊需结合临床病史与组织病理学综合考虑。其发生机制,目前还无较明确而有力的证实。文中就该病的流行病学特征、临床病理特点及免疫表型、发生机制等进行综述。
Nephrotic systemic fibrosis (NSF) is a systemic sclerosis that occurs in patients with renal insufficiency due to injection of MRI contrast agents and involves multiple organs throughout the body, but the lower limbs are the first. Initial symptoms of skin erythema, stiffness, progression of the disease involving multiple organs throughout the body, and eventually died of organ failure. Skin biopsy showed a large number of collagen dermis and fibrous tissue hyperplasia, combined diagnosis of clinical history and histopathology should be considered. Its mechanism, there is no more clear and strong confirmation. The epidemiological characteristics of the disease, clinical and pathological features and immunophenotype, mechanism of occurrence are reviewed.