An Unusual Case of a Pulmonary-Renal Syndrome with Negative Anti-GBM and ANCA Antibodies

• 作者: Edson Ricardo Junior Emanuel Malaguez Webber Fabrício Malaguez Webber Maria Belen López Morillo
• 来源期刊: 临床医学病理报告(英文)
• 学科: 医学
• 关键词: PULMONARY RENAL Syndrome Goodpasture ANCA Anti-GBM
• 年，卷（期）: 2018,（8）
• 页码范围: 461-469
• 页数: 9页
• 分类号: R5

摘要

Pulmonary renal syndrome (PRS) is a high mortality, rare disorder presenting with diffuse alveolar hemorrhage and progressive acute glomerulonephritis. This syndrome is often caused by autoimmune entities, the most frequent being ANCA positive vasculitis and anti-GBM disease. We report a case of a 34-year-old Chilean woman, who initially presents with anemia and after a few days of inpatient management, starts with progressively worsening dyspnea, decrease in renal function and hematuria. The patient is initially diagnosed with pneumonia, but further evaluation using Thorax CT scan and renal biopsy confirms the suspicion of PRS. The case is of particular interest due to the lack of extensive bibliography on anti-GBM and ANCA negative pulmonary-renal syndrome, an uncategorized subtype of this syndrome with unknown optimal management.