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伴有肾脏异常和抗肾小球基底膜自身抗体迟发表现的古德帕斯彻综合征

Goodpasture's disease with late presentation of renal abnormality and anti-GBM autoantibody.

作者信息

Boardman Emily Ann, Sohail Sameira, Yadavilli Rajesh

机构信息

Cardiff University, Cardiff, South Glamorgan, UK.

Royal Bolton Hospital NHS Foundation Trust, Bolton, UK.

出版信息

BMJ Case Rep. 2017 Mar 17;2017:bcr2016218705. doi: 10.1136/bcr-2016-218705.

DOI:10.1136/bcr-2016-218705
PMID:28314809
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5372237/
Abstract

Goodpasture's disease without circulating autoantibodies is a rare presentation of a rare diagnosis. We present the case of a man aged 17 years who had 3 hospital admissions over a 2-month period with haemoptysis and shortness of breath. Throughout his first 2 admissions, his renal function was normal and anti-glomerular basement membrane (GBM) antibodies were negative. CT pulmonary angiogram during his second admission revealed florid and diffuse alveolar infiltrates. However, high-resolution CT chest performed 4 weeks later showed complete resolution of these changes. On his third admission, he developed acute kidney injury. A repeat CT chest revealed the reappearance of initial findings and anti-GBM antibodies were now positive. Goodpasture's disease was subsequently confirmed with renal biopsy. Our case, with delayed onset of renal impairment, initial seronegativity for anti-GBM antibodies and relapsing and remitting CT findings, emphasises the need to consider this diagnosis in the setting of otherwise unexplained pulmonary haemorrhage.

摘要

无循环自身抗体的肺出血肾炎综合征是一种罕见诊断的罕见表现。我们报告一例17岁男性病例,该患者在2个月内因咯血和呼吸急促3次入院。在其前两次入院期间,他的肾功能正常,抗肾小球基底膜(GBM)抗体呈阴性。第二次入院时的CT肺动脉造影显示有明显的弥漫性肺泡浸润。然而,4周后进行的胸部高分辨率CT显示这些改变完全消退。第三次入院时,他出现了急性肾损伤。重复胸部CT显示最初的表现再次出现,此时抗GBM抗体呈阳性。随后通过肾活检确诊为肺出血肾炎综合征。我们的病例,肾功能损害出现延迟,抗GBM抗体最初呈血清阴性,CT表现复发和缓解,强调在不明原因的肺出血情况下需要考虑这一诊断。

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Goodpasture's syndrome: a case of delayed appearance of autoantibodies and renal disease.古德帕斯丘综合征:自身抗体及肾脏疾病延迟出现的一例病例
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