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硬皮病合并干燥综合征:一例肾脏急症病例报告

Scleroderma Overlapping With Sjögren's Syndrome: A Case Report of a Renal Emergency.

作者信息

Mumajesi Suela, Seferi Saimir, Spahia Nereida, Gjecka Elena, Kurti Floreta

机构信息

Nephrology, University Hospital Center "Mother Tereza", Tirana, ALB.

Gastroenterology and Hepatology, University Hospital Center "Mother Tereza", Tirana, ALB.

出版信息

Cureus. 2025 Jun 12;17(6):e85861. doi: 10.7759/cureus.85861. eCollection 2025 Jun.

Abstract

Scleroderma renal crisis (SRC) is the most common hallmark of renal involvement in systemic sclerosis (SSc), characterized by acute kidney injury and malignant hypertension. The pathophysiology of SRC involves endothelial dysfunction, activation of the renin-angiotensin-aldosterone system (RAAS), and thrombotic microangiopathy (TMA), leading to renal ischemia and hypertension. When accompanied by overlapping Sjögren's syndrome (SS), the clinical presentation and therapy become considerably more complex. We discuss the diagnosis, management, and outcomes of a patient presenting with SRC and overlapping SS. It highlights the importance of early detection and rapid treatment of SRC, particularly in the presence of overlap syndromes. Early intervention with angiotensin-converting enzyme (ACE) inhibitors and careful monitoring of renal function are crucial for optimal outcomes. The occurrence of overlapping SS complicates the clinical presentation and therapy, emphasizing the importance of an individualized approach to treatment. Healthcare personnel must be especially mindful of this since early detection of renal crisis has a major impact on survival rates and long-term prognosis.

摘要

硬皮病肾危象(SRC)是系统性硬化症(SSc)肾脏受累最常见的特征,其特点是急性肾损伤和恶性高血压。SRC的病理生理学涉及内皮功能障碍、肾素-血管紧张素-醛固酮系统(RAAS)激活和血栓性微血管病(TMA),导致肾缺血和高血压。当合并重叠综合征干燥综合征(SS)时,临床表现和治疗会变得更加复杂。我们讨论了一名患有SRC和重叠SS患者的诊断、管理及预后。它强调了早期发现和快速治疗SRC的重要性,尤其是在存在重叠综合征的情况下。早期使用血管紧张素转换酶(ACE)抑制剂进行干预并仔细监测肾功能对于获得最佳预后至关重要。重叠SS的出现使临床表现和治疗变得复杂,强调了个体化治疗方法的重要性。医护人员必须特别注意这一点,因为早期发现肾危象对生存率和长期预后有重大影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9004/12255470/67d1933a4b2a/cureus-0017-00000085861-i01.jpg

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