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系统性硬皮病中的呼吸衰竭。

Respiratory failure in systemic sclerosis.

机构信息

Division of Rheumatology, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Av. Dr. Arnaldo, 455, 3O. Andar, Cerqueira César, Sao Paulo, SP, CEP: 01246-903, Brazil.

出版信息

Rheumatol Int. 2024 Nov;44(11):2653-2658. doi: 10.1007/s00296-023-05482-4. Epub 2023 Oct 14.

Abstract

Systemic sclerosis (SSc) can lead to dyspnea and respiratory failure through multiple mechanisms, making a precise diagnosis particularly challenging, especially amid the current COVID-19 pandemic. In this report, we present a case involving a 26-year-old female who had previously undiagnosed SSc. She experienced acute respiratory failure necessitating orotracheal intubation. Following an extensive evaluation, the patient exhibited skin thickening, kidney failure, thrombocytopenia, microangiopathic anemia, and an antinuclear antibody with a nuclear fine speckled pattern at a titer of 1:320. A diagnosis of SSc complicated by scleroderma renal crisis (SRC) was established. The patient's condition improved after undergoing hemodialysis, receiving an angiotensin-converting enzyme inhibitor, and undergoing cyclophosphamide treatment. Subsequently, she demonstrated sustained improvement during a follow-up period of 20 months.

摘要

系统性硬化症(SSc)可通过多种机制导致呼吸困难和呼吸衰竭,使得准确诊断变得极具挑战性,尤其是在当前 COVID-19 大流行期间。在本报告中,我们介绍了一例 26 岁的女性病例,她此前未被诊断出患有 SSc,出现急性呼吸衰竭需要进行经口气管插管。经过广泛评估,患者表现出皮肤增厚、肾衰竭、血小板减少、微血管性溶血性贫血以及抗核抗体呈核细颗粒状阳性,滴度为 1:320。诊断为并发硬皮病肾危象(SRC)的 SSc。该患者在接受血液透析、血管紧张素转换酶抑制剂和环磷酰胺治疗后,病情得到改善。随后,在 20 个月的随访期间,她的病情持续改善。

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