关键词: anti-GBM disease pulmonary haemorrhage rapidly progressive glomerulonephritis

来  源:   DOI:10.31138/mjr.34.2.256   PDF(Pubmed)

Abstract:
Anti-GBM disease is a rare, life-threatening small vessel vasculitis caused by circulating anti-GBM antibodies resulting to rapidly progressive glomerulonephritis and/or pulmonary haemorrhage. The gold standard for the diagnosis is the renal biopsy with the pathognomonic finding of linear deposition of IgG along the glomerular capillaries. Early diagnosis and intervention are key determinants of the response to therapy and long-term prognosis of these patients. However, during COVID-19 pandemic recognizing a pulmonary-renal syndrome caused by autoimmune diseases has become challenging. Herein, we aimed to describe a rare case of anti-GBM disease with pulmonary haemorrhage and rapidly progressive glomerulonephritis in a young man in a tertiary referral hospital in Greece, while COVID-19 pandemic was at its peak. Although the patient presented high level of creatinine and crescents, the early diagnosis and start of treatment resulted to favourable renal prognosis.
摘要:
抗GBM疾病是一种罕见的,循环抗GBM抗体引起的危及生命的小血管血管炎,导致快速进行性肾小球肾炎和/或肺出血。诊断的金标准是肾活检,其病理发现是IgG沿肾小球毛细血管线性沉积。早期诊断和干预是这些患者对治疗反应和长期预后的关键决定因素。然而,在COVID-19大流行期间,认识到由自身免疫性疾病引起的肺-肾综合征已变得具有挑战性。在这里,我们的目的是描述一个罕见的抗GBM疾病,肺出血和快速进展性肾小球肾炎的一个年轻人在希腊三级转诊医院,而COVID-19疫情处于高峰。尽管患者出现高水平的肌酐和新月,早期诊断和开始治疗导致良好的肾脏预后.
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