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Goodpasture’s Disease: An Uncommon Disease With an Atypical Clinical Course

机译:Goodpasture病:具有非典型临床过程的罕见疾病

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Goodpasture’s disease is an uncommon composite of features including renal failure with pulmonary hemorrhage secondary to an autoimmune response that specifically targets these organ systems. We present a case of particular interest in regards to atypical presentation, and the uncommon treatment that the patient underwent. A 65-year-old Afghani female arrived with complaints of nausea, vomiting, loss of appetite, malaise, decreased urine output, exertional dyspnea, and cough. The patient presented initially with renal failure and unexpectedly developed respiratory failure after hemodialysis. Initial CT of thorax revealed diffuse bilateral pulmonary edema. Subsequently, the patient received a bronchoscopy demonstrating alveolar hemorrhage, which highlights a clinician’s need to maintain a differential and reassess patients. Anti-GBM antibody in the serum was detected and the renal biopsy revealed evidence of the antibody on immunofluorescence. In regards to management, the patient could only be treated with plasmapheresis as she had contraindication to initiation of immunosuppression, after which she showed significant clinical improvement. We would like to highlight the benefit of plasmapheresis without concomitant immunosuppression and recommend such an approach to be considered in similar clinical scenarios, where contraindication for immunosuppressant therapy exists.J Clin Med Res. 2016;8(1):52-55doi: http://dx.doi.org/10.14740/jocmr2379w
机译:Goodpasture病是一种罕见的综合性疾病,其特征包括肾功能衰竭和继发于针对这些器官系统的自身免疫反应继发的肺出血。我们针对非典型表现以及患者接受的罕见治疗提出了一个特别令人感兴趣的案例。一名65岁的阿富汗裔女性因恶心,呕吐,食欲不振,不适,尿量减少,劳累性呼吸困难和咳嗽而来。患者最初表现为肾衰竭,血液透析后意外出现呼吸衰竭。胸部CT扫描显示双侧弥漫性肺水肿。随后,患者接受了表明肺泡出血的支气管镜检查,这突显了临床医生需要保持差异并重新评估患者。检测到血清中的抗GBM抗体,肾脏活检显示该抗体具有免疫荧光的证据。在管理方面,患者只能接受血浆置换术,因为她有开始免疫抑制的禁忌症,此后她表现出明显的临床改善。我们想强调血浆置换术在不伴随免疫抑制的同时的好处,并建议在存在免疫抑制剂治疗禁忌症的类似临床情况下考虑采用这种方法。 2016; 8(1):52-55doi:http://dx.doi.org/10.14740/jocmr2379w

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