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一例由肺出血肾炎综合征引发的心肾综合征的独特病例。

A Unique Case of Goodpasture's Syndrome-Induced Cardiorenal Syndrome.

作者信息

Chinniah Chidambaram, Pyronneau Alexander, Stepman Gauthier, Ali Rias

机构信息

Cardiology, HCA Healthcare/University of South Florida Morsani College of Medicine, Graduate Medical Education at HCA Florida Trinity Hospital, Trinity, USA.

Internal Medicine, HCA Healthcare/University of South Florida Morsani College of Medicine, Graduate Medical Education at HCA Florida Trinity Hospital, Trinity, USA.

出版信息

Cureus. 2024 Jul 10;16(7):e64269. doi: 10.7759/cureus.64269. eCollection 2024 Jul.

Abstract

Goodpasture's syndrome (GPS) is a rare small vessel vasculitis characterized by circulating antibodies directed against the glomerular and alveolar basement membrane leading to renal and pulmonary manifestations. Here, we discuss a unique case of a 30-year-old Caucasian male smoker initially presenting with hemoptysis and anemia who was found to have biopsy-proven GPS with elevated anti-glomerular basement membrane (anti-GBM) antibodies. Unfortunately, the patient failed four months of standard treatment for GPS leading to end-stage renal disease (ESRD), while uniquely developing cardiorenal syndrome (CRS) with non-ischemic cardiomyopathy resulting in systolic and diastolic heart failure (HF). Despite aggressive medical management and hemodialysis, the patient's cardiac function continued to decline and the decision was made to insert an automatic implantable cardioverter defibrillator (AICD). To our knowledge, this is the first reported case of an anti-GBM-positive GPS patient who developed dilated cardiomyopathy. The importance of this report is to illustrate the rarity of developing CRS with non-ischemic cardiomyopathy and congestive heart failure from GPS and highlight the difficulty of determining management changes beyond guideline-directed medical therapy (GDMT) in GPS to slow the progression of worsening cardiac function.

摘要

肺出血肾炎综合征(GPS)是一种罕见的小血管炎,其特征是循环抗体针对肾小球和肺泡基底膜,导致肾脏和肺部表现。在此,我们讨论一例独特病例,一名30岁的白种男性吸烟者,最初表现为咯血和贫血,经活检证实患有GPS,抗肾小球基底膜(抗GBM)抗体升高。不幸的是,该患者接受了四个月的GPS标准治疗但失败,导致终末期肾病(ESRD),同时独特地发展为心肾综合征(CRS),伴有非缺血性心肌病,导致收缩性和舒张性心力衰竭(HF)。尽管进行了积极的药物治疗和血液透析,患者的心脏功能仍持续下降,因此决定植入自动植入式心脏复律除颤器(AICD)。据我们所知,这是首例报道的抗GBM阳性GPS患者发生扩张型心肌病的病例。本报告的重要性在于说明GPS并发非缺血性心肌病和充血性心力衰竭的心肾综合征的罕见性,并强调在GPS中确定超出指南指导的药物治疗(GDMT)以减缓心脏功能恶化进展的管理变化的困难。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/74b7/11236434/74baebd1db10/cureus-0016-00000064269-i01.jpg

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