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揭示交集:硬皮病肾危象并发膜性肾病

Revealing the Intersection: Scleroderma Renal Crisis Complicating Membranous Nephropathy.

作者信息

Kazi Ashar Uddin, Kibria Saifuddin Mohammad, Rahman Shipi Monowara, Ahmed Rameen, Mustafa Rafid

机构信息

Internal Medicine, United Lincolnshire Teaching Hospitals NHS Trust, Lincoln, GBR.

Medicine, Lincoln Medical School, University of Nottingham, Lincoln, GBR.

出版信息

Cureus. 2025 May 1;17(5):e83327. doi: 10.7759/cureus.83327. eCollection 2025 May.

Abstract

An 82-year-old woman with systemic sclerosis (anti-topoisomerase I (anti-Scl-70) positive) and a history of membranous nephropathy presented with a prolonged illness marked by worsening oedema, reduced urine output, and severe hypertension. Laboratory investigations revealed nephrotic-range proteinuria, acute kidney injury (AKI), and a marked decline in renal function. Initial treatment with diuretics and antihypertensive therapy yielded limited improvement. Renal biopsy demonstrated dual pathology: primary membranous nephropathy and acute vascular changes indicative of scleroderma renal crisis (SRC). Despite targeted therapy, the patient experienced flash pulmonary oedema, necessitating haemodialysis. This case highlights the complexities of diagnosing and managing overlapping renal pathologies in systemic sclerosis, underscoring the critical importance of early recognition and intervention in SRC.

摘要

一名82岁女性,患有系统性硬化症(抗拓扑异构酶I(抗Scl - 70)阳性)且有膜性肾病病史,此次因病程迁延就诊,表现为水肿加重、尿量减少和严重高血压。实验室检查显示肾病范围蛋白尿、急性肾损伤(AKI)以及肾功能显著下降。利尿剂和抗高血压治疗的初始治疗效果有限。肾活检显示双重病理:原发性膜性肾病和提示硬皮病肾危象(SRC)的急性血管改变。尽管进行了靶向治疗,患者仍发生了急性肺水肿,需要进行血液透析。该病例突出了系统性硬化症中重叠肾病理诊断和管理的复杂性,强调了SRC早期识别和干预的至关重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6164/12127705/52967e9f9347/cureus-0017-00000083327-i01.jpg

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