PDF(Pubmed)
Abstract：
Anti-glomerular basement membrane disease (anti-GBM disease) associated with renal and lung lesions has a poor prognosis. Diffuse alveolar hemorrhage (DAH) is a complication that worsens anti-GBM disease prognosis. We report a rescue case using veno-venous extracorporeal membrane oxygenation (VV-ECMO) for diffuse alveolar hemorrhage due to isolated pulmonary anti-GBM disease; a rare anti-GBM syndrome. A 30-year-old Japanese female with no past medical history. Presented with acute hypoxemic respiratory failure requiring mechanical ventilation. Progressive deterioration and refractory hypoxemia prompted therapy with VV-ECMO. Serum anti-GBM antibody confirmed the diagnosis of anti-GBM disease. Multi-modal systemic therapy with pulse-dosed methylprednisolone, plasma exchange, and rituximab resulted in significant clinical improvement. VV-ECMO for 10 days was uncomplicated. Renal replacement therapy was not required. The patient was extubated on day 18 and discharged from the hospital after 45 days. VV-ECMO supportive therapy for DAH with refractory respiratory failure was demonstrated to be effective pending definitive diagnostic and therapeutic management in this case of isolated pulmonary anti-GBM disease.
摘要：
与肾脏和肺部病变相关的抗肾小球基底膜疾病（抗GBM疾病）预后不良。弥漫性肺泡出血（DAH）是一种恶化抗GBM疾病预后的并发症。我们报告了一例使用静脉-静脉体外膜氧合（VV-ECMO）治疗孤立性肺抗GBM疾病引起的弥漫性肺泡出血的抢救病例；一种罕见的抗GBM综合征。一名30岁的日本女性，没有既往病史。表现为急性低氧性呼吸衰竭，需要机械通气。进行性恶化和难治性低氧血症促使VV-ECMO治疗。血清抗GBM抗体证实了抗GBM疾病的诊断。脉冲剂量甲基强的松龙的多模式全身治疗，血浆置换,和利妥昔单抗导致显著的临床改善.VV-ECMO治疗10天无并发症。不需要肾脏替代疗法。患者在第18天拔管，45天后出院。在这种孤立的肺抗GBM疾病的情况下，经过明确的诊断和治疗管理，VV-ECMO支持治疗难治性呼吸衰竭的DAH被证明是有效的。
