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系统性硬化症合并心脏压塞及硬皮病肾危象:一例报告

Cardiac tamponade with scleroderma renal crisis in systemic sclerosis: a case report.

作者信息

Shrestha Suraj, Aryal Roshan, Acharya Suman, Shrestha Khagendra, Twayana Anuradha

机构信息

Maharajgunj Medical Campus, Institute of Medicine, Kathmandu, Nepal.

Department of Internal Medicine, Tribhuvan University Teaching Hospital, Kathmandu, Nepal.

出版信息

Ann Med Surg (Lond). 2025 Aug 5;87(9):6163-6167. doi: 10.1097/MS9.0000000000003646. eCollection 2025 Sep.

Abstract

INTRODUCTION AND IMPORTANCE

Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis of internal organs and vasculopathy. It commonly involves the skin and multiple organs, including the kidneys and cardiovascular system. Cardiac tamponade in SSc is an uncommon manifestation, often occurring alongside scleroderma renal crisis (SRC).

CASE PRESENTATION

A 50-year-old woman presented with sudden shortness of breath, chest pain, and reduced urine output. Diagnostic tests revealed a large pericardial effusion leading to cardiac tamponade, acute kidney injury, and microangiopathic hemolytic anemia. Clinical assessment and immunological testing confirmed the diagnosis of systemic sclerosis. The patient received pericardiocentesis, hemodialysis, and was treated with diuretics and angiotensin-converting enzyme inhibitors (ACEIs).

DISCUSSION

Pericardial effusion is frequently seen in SSc, but it is difficult to determine whether it directly contributes to or results from SRC. Early diagnosis and the use of ACEIs have shown promising benefits in managing SRC.

CONCLUSION

This case underscores the rare yet critical presentation of SSc with concurrent cardiac tamponade and SRC, emphasizing the importance of early diagnosis and multidisciplinary management.

摘要

引言与重要性

系统性硬化症(SSc)是一种罕见的自身免疫性疾病,其特征为内脏器官纤维化和血管病变。它通常累及皮肤和多个器官,包括肾脏和心血管系统。系统性硬化症中的心脏压塞是一种不常见的表现,常与硬皮病肾危象(SRC)同时发生。

病例介绍

一名50岁女性出现突发气短、胸痛和尿量减少。诊断检查显示大量心包积液导致心脏压塞、急性肾损伤和微血管病性溶血性贫血。临床评估和免疫学检测确诊为系统性硬化症。患者接受了心包穿刺术、血液透析,并接受了利尿剂和血管紧张素转换酶抑制剂(ACEIs)治疗。

讨论

心包积液在系统性硬化症中很常见,但很难确定它是直接导致硬皮病肾危象还是由其引起。早期诊断和使用血管紧张素转换酶抑制剂在管理硬皮病肾危象方面已显示出有前景的益处。

结论

本病例强调了系统性硬化症并发心脏压塞和硬皮病肾危象这种罕见但关键的表现,强调了早期诊断和多学科管理的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/053d/12401429/d65b83cec99b/ms9-87-6163-g001.jpg

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