Malho Anabela, Santos Viriato, Cabrita Ana, Silva Ana Paula, Pinto Isabel, Bernardo Idalécio, Neves Pedro Leão
Serviço de Nefrologia, Hospital de Faro, Rua Leão Penedo, 8000 Faro, Portugal.
Int J Nephrol. 2010 Aug 16;2010:383548. doi: 10.4061/2010/383548.
Goodpasture's disease is a rare autoimmune disorder characterised by the development of antiglomerular basement membrane autoantibodies, which typically presents with rapidly progressive crescentic glomerulonephritis and pulmonary haemorrhage. Even with aggressive nonspecific immunosuppression and plasma exchange, mortality remains high. We report a case of life-threatening Goodpasture's disease with relapsing pulmonary haemorrhage refractory to conventional therapy. Second line treatment was based on mycophenolate mofetil 1 g every 12 hours and prednisolone 60 mg/day. In this case, the use of a low-dose mycophenolate mofetil regimen turned out to be insufficient. However, in our opinion higher mycophenolate mofetil doses should be considered an alternative treatment, mainly in relapsing disease, due to its mechanism of action and current insufficient therapeutic weapons.
肺出血肾炎综合征是一种罕见的自身免疫性疾病,其特征是抗肾小球基底膜自身抗体的产生,通常表现为快速进展的新月体性肾小球肾炎和肺出血。即使采用积极的非特异性免疫抑制和血浆置换治疗,死亡率仍然很高。我们报告一例危及生命的肺出血肾炎综合征病例,该患者复发性肺出血,常规治疗无效。二线治疗方案为霉酚酸酯1克每12小时一次和泼尼松龙60毫克/天。在本病例中,低剂量霉酚酸酯治疗方案被证明是不足的。然而,我们认为,由于霉酚酸酯的作用机制以及目前治疗手段的不足,更高剂量的霉酚酸酯应被视为一种替代治疗方法,主要用于复发性疾病。