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Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease resulting in dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition

机译:尽管血管紧张素转换酶被抑制,但新诊断为混合性结缔组织病的硬皮病肾病仍导致依赖透析的慢性肾脏疾病

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摘要

Mixed connective tissue disease (MCTD) is a rheumatic disease with a combination of multiple connective tissue disorders, which includes dermatomyositis or polymyositis, systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis and Sjögren’s syndrome. It affects various organs of the body, which includes the lungs, heart, kidneys, joints, muscles and the haematological system. Here, we report a case of MCTD consisting of scleroderma, Sjögren’s syndrome and polymyositis complicated by scleroderma renal crisis (SRC) but with negative anti-nuclear antibody (ANA), anti-Scl 70 and anti-centromere antibodies. The patient was started on captopril for the treatment of SRC but developed chronic kidney disease despite adequate blood pressure control with angiotensin-converting enzyme inhibitor (ACEi).
机译:混合性结缔组织病(MCTD)是一种风湿病,合并了多种结缔组织疾病,包括皮肌炎或多发性肌炎,系统性硬化症,系统性红斑狼疮,类风湿性关节炎和干燥综合征。它影响身体的各个器官,包括肺,心脏,肾脏,关节,肌肉和血液系统。在这里,我们报告了一例由硬皮病,干燥综合征和多发性肌炎并发硬皮肾病(SRC)的MCTD病例,但其抗核抗体(ANA),抗Scl 70和抗着丝粒抗体呈阴性。该患者开始使用卡托普利治疗SRC,但尽管通过血管紧张素转换酶抑制剂(ACEi)适当控制了血压,但仍发展为慢性肾脏疾病。

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