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肺肾综合征:诊断挑战

Pulmonary-renal syndrome: diagnostic challenge.

作者信息

Correia Sandra Isabel, Eira Isabel, Santa Cruz Andre, Ângela Cristina

机构信息

Internal Medicine, Hospital of Braga, Braga, Portugal.

出版信息

BMJ Case Rep. 2019 Apr 25;12(4):e227720. doi: 10.1136/bcr-2018-227720.

Abstract

Vasculitis presents several diagnostic challenges. Herein, we present a clinical case of a 71- year old woman, observed in our emergency department due to asthenia, vomiting and persistent cough. The patient had a history of progressive renal failure and anaemia over the last years. On physical examinations, fine pulmonary crackles were detected and laboratory test showed haemoglobin 69 g/L, creatinine 4 mg/dL, potassium 6.3 mmol/L, positive antineutrophil cytoplasm antibody (ANCA), with proteins and dimorphic erythrocytes in the urinary sediment. CT analysis of the thorax revealed patchy ground glass haziness, likely due to diffuse alveolar haemorrhage. Pulmonary-renal syndrome was assumed, and induction therapy was initiated. She was discharged after 33 days of hospitalisation. On the following months, ANCA titres remained undetectable, but minor recovery of renal function was observed, requiring haemodialysis. Indeed, the use of aggressive induction therapy at early stage dramatically improve prognosis, maintenance of disease remission may be difficult, as relapse is frequent.

摘要

血管炎存在诸多诊断挑战。在此,我们呈现一例71岁女性的临床病例,该患者因乏力、呕吐和持续咳嗽在我们急诊科就诊。患者在过去几年中有进行性肾衰竭和贫血病史。体格检查时,发现肺部有细湿啰音,实验室检查显示血红蛋白69g/L、肌酐4mg/dL、钾6.3mmol/L、抗中性粒细胞胞浆抗体(ANCA)阳性,尿沉渣中有蛋白和异形红细胞。胸部CT分析显示斑片状磨玻璃样模糊影,可能是由于弥漫性肺泡出血所致。考虑为肺肾综合征,并开始诱导治疗。住院33天后出院。在随后的几个月里,ANCA滴度检测不到,但观察到肾功能有轻微恢复,仍需要血液透析。事实上,早期使用积极的诱导治疗可显著改善预后,但由于疾病频繁复发,维持疾病缓解可能较为困难。

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本文引用的文献

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Goodpasture's syndrome: a clinical update.Goodpasture 综合征:临床更新。
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