PDF(Pubmed)
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：
Goodpasture综合征（GPS）是一种罕见的小血管血管炎，其特征是针对肾小球和肺泡基底膜的循环抗体导致肾脏和肺部表现。这里,我们讨论了一例30岁白人男性吸烟者的独特病例,最初出现咯血和贫血,他被发现具有活检证实的GPS和升高的抗肾小球基底膜(抗GBM)抗体.不幸的是,患者未能通过四个月的GPS标准治疗导致终末期肾病（ESRD），而独特的发展心肾综合征（CRS）与非缺血性心肌病导致收缩和舒张性心力衰竭（HF）。尽管积极的医疗管理和血液透析，患者的心脏功能持续下降,因此决定插入自动植入式心律转复除颤器(AICD).据我们所知,这是首例报道的发生扩张型心肌病的抗GBM阳性GPS患者.本报告的重要性是为了说明GPS引起的非缺血性心肌病和充血性心力衰竭的CRS的罕见性，并强调难以确定GPS中超出指南指导的药物治疗（GDMT）的管理变化以减缓心脏恶化的进展功能。
