de Groot K, Schnabel A
Abteilung für Nephrologie, Medizinische Hochschule Hannover.
Internist (Berl). 2005 Jul;46(7):769-81; quiz 782. doi: 10.1007/s00108-005-1423-8.
Pulmonary-renal syndrome is a potentially life-threatening disorder, characterized by diffuse alveolar hemorrhage on the basis of pulmonary capillaritis in conjunction with rapidly progressive glomerulonephritis. Pulmonary-renal syndrome can originate from various systemic autoimmune diseases. ANCA-associated vasculitides account for approximately 60%, Goodpasture's Syndrome for approximately 20% of the cases. Fulminant pulmonary capillaritis can result in acute respiratory failure, more subtle courses are only detected by bronchoalveolar lavage. Renal biopsy displays extracapillary proliferating glomerulonephritis and renal immunohistology facilitates detection of the underlying systemic disease. By accelerating the diagnosis of the specific underlying disease, autoantibody testing fosters rapid initiation of treatment and thereby significantly improved the prognosis of pulmonary-renal syndrome. Intense immunosuppression with cyclophosphamide and glucocorticoids, augmented by plasmapheresis in the event of Goodpastures' syndrome, is the mainstay of therapy. Supportive measures as temporary ventilation and hemodialysis have further reduced mortality.
肺肾综合征是一种潜在的危及生命的疾病，其特征是在肺毛细血管炎的基础上出现弥漫性肺泡出血，并伴有快速进展性肾小球肾炎。肺肾综合征可源于各种全身性自身免疫性疾病。抗中性粒细胞胞浆抗体（ANCA）相关血管炎约占病例的60%，Goodpasture综合征约占20%。暴发性肺毛细血管炎可导致急性呼吸衰竭，更隐匿的病程仅通过支气管肺泡灌洗才能检测到。肾活检显示毛细血管外增生性肾小球肾炎，肾脏免疫组织学有助于检测潜在的全身性疾病。通过加速对特定潜在疾病的诊断，自身抗体检测促进了治疗的快速启动，从而显著改善了肺肾综合征的预后。以环磷酰胺和糖皮质激素进行强化免疫抑制，在Goodpasture综合征的情况下辅以血浆置换，是主要的治疗方法。诸如临时通气和血液透析等支持性措施进一步降低了死亡率。
