Daimon S, Umeda T, Michishita I, Wakasugi H, Genda A, Koni I
Yokohame Sakae Kyosai Hospital.
Intern Med. 1994 Sep;33(9):569-73. doi: 10.2169/internalmedicine.33.569.
A 49-year-old man was admitted because of general fatigue, cough and hematuria. During the hospital course, acute renal failure, hemoptysis and dyspnea developed. A percutaneous renal biopsy revealed a diffuse crescentic glomerulonephritis, and direct immunofluorescence showed a linear pattern of IgG along the glomerular basement membrane. Although serum anti-glomerular basement membrane (anti-GBM) antibody was not detected. Goodpasture's-like syndrome was suspected, and methylprednisolone pulse therapy and plasmapheresis were administered. Concomitantly, extracorporeal membrane oxygenation (ECMO) was instituted because of deterioration in respiratory status due to a severe pulmonary hemorrhage despite maximal ventilatory support. Temporarily, the patient improved and ECMO was discontinued. ECMO may be a useful therapeutic support for hypoxia resulting from pulmonary hemorrhage in Goodpasture's syndrome (GPS) and Goodpasture's-like syndrome.
一名49岁男性因全身乏力、咳嗽和血尿入院。在住院期间，出现了急性肾衰竭、咯血和呼吸困难。经皮肾活检显示为弥漫性新月体性肾小球肾炎，直接免疫荧光显示沿肾小球基底膜有IgG线性沉积。尽管未检测到血清抗肾小球基底膜（anti-GBM）抗体，但仍怀疑为类Goodpasture综合征，并给予甲泼尼龙冲击治疗和血浆置换。同时，由于尽管给予了最大程度的通气支持，但仍因严重肺出血导致呼吸状况恶化，故开始进行体外膜肺氧合（ECMO）治疗。患者病情暂时改善，ECMO停用。ECMO可能是治疗Goodpasture综合征（GPS）和类Goodpasture综合征中肺出血所致缺氧的有用治疗支持手段。
