Tashiro Hiroki, Takahashi Koichiro, Ikeda Yuki, Uchiumi Saori, Fukuda Makoto, Motoaki Miyazono, Kimura Shinya, Sueoka-Aragane Naoko
Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan.
Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga, Japan.
J Med Case Rep. 2017 Apr 13;11(1):102. doi: 10.1186/s13256-017-1273-8.
Goodpasture's syndrome is a rare disease that is characterized by rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage.
A 71-year-old Japanese man who had chronic interstitial pneumonia was diagnosed as having Goodpasture's syndrome. Both anti-glomerular basement membrane antibody and myeloperoxidase anti-neutrophil cytoplasmic antibody were increased. Despite intensive treatments, including mechanical ventilation, he died from respiratory failure. Pathological findings at autopsy showed rapidly progressive glomerulonephritis in his kidneys, diffuse alveolar hemorrhage, hyaline membranes, and fibroblastic foci in his lungs. The cause of death was diagnosed as respiratory failure as a result of diffuse alveolar damage induced by a combination of diffuse alveolar hemorrhage and exacerbation of interstitial pneumonia.
We report a case of Goodpasture's syndrome complicated with pre-existing chronic interstitial pneumonia and positive myeloperoxidase anti-neutrophil cytoplasmic antibody. We reviewed six similar cases reported in the literature and concluded that Goodpasture's syndrome with pre-existing interstitial pneumonia and myeloperoxidase anti-neutrophil cytoplasmic antibody is related to a poor prognosis.
肺出血肾炎综合征是一种罕见疾病，其特征为快速进展性肾小球肾炎和弥漫性肺泡出血。
一名71岁患有慢性间质性肺炎的日本男性被诊断为肺出血肾炎综合征。抗肾小球基底膜抗体和髓过氧化物酶抗中性粒细胞胞浆抗体均升高。尽管接受了包括机械通气在内的强化治疗，他仍死于呼吸衰竭。尸检病理结果显示其肾脏有快速进展性肾小球肾炎，肺部有弥漫性肺泡出血、透明膜和成纤维细胞灶。死亡原因诊断为弥漫性肺泡出血和间质性肺炎加重共同导致的弥漫性肺泡损伤引起的呼吸衰竭。
我们报告一例合并既往慢性间质性肺炎且髓过氧化物酶抗中性粒细胞胞浆抗体阳性的肺出血肾炎综合征病例。我们回顾了文献中报道的6例类似病例，并得出结论，合并既往间质性肺炎和髓过氧化物酶抗中性粒细胞胞浆抗体的肺出血肾炎综合征与预后不良有关。
