Martin Kirsten, Deleveaux Spencer, Cunningham Marcus, Ramaswamy Kavitha, Thomas Beje, Lerma Edgar, Madariaga Hector
Medstar Georgetown University Hospital, United States.
Advocate Christ Medical Center, United States.
Dis Mon. 2022 Dec;68(12):101465. doi: 10.1016/j.disamonth.2022.101465. Epub 2022 Aug 23.
Pulmonary renal syndrome (PRS) is a constellation of different disorders that cause both rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. While antineutrophil cytoplasmic antibody associated vasculitis and anti-glomerular basement membrane disease are the predominant causes of PRS, numerous other mechanisms have been shown to cause this syndrome, including thrombotic microangiopathies, drug exposures, and infections, among others. This syndrome has high morbidity and mortality, and early diagnosis and treatment is imperative to improve outcomes. Treatment generally involves glucocorticoids and immunosuppressive agents, but treatment targeted to the underlying disorder can improve outcomes and mitigate side effects. Familiarity with the wide range of possible causes of PRS can aid the clinician in workup, diagnosis and early initiation of treatment. This review provides a summary of the clinical presentation, etiologies, pathophysiology, and treatment of PRS.
肺肾综合征（PRS）是一组不同的疾病，可导致快速进展性肾小球肾炎和弥漫性肺泡出血。虽然抗中性粒细胞胞浆抗体相关性血管炎和抗肾小球基底膜病是PRS的主要病因，但已证明许多其他机制也可导致该综合征，包括血栓性微血管病、药物暴露和感染等。该综合征具有高发病率和死亡率，早期诊断和治疗对于改善预后至关重要。治疗通常包括糖皮质激素和免疫抑制剂，但针对潜在疾病的治疗可改善预后并减轻副作用。熟悉PRS的各种可能病因有助于临床医生进行检查、诊断和早期治疗。本综述总结了PRS的临床表现、病因、病理生理学和治疗方法。
