Drew P J, Newland A C, Marsh F P
Postgrad Med J. 1984 Jan;60(699):52-5. doi: 10.1136/pgmj.60.699.52.
A 16-year-old boy developed an immune complex illness associated with lung haemorrhage, proliferative nephritis with crescents and renal failure. Treatment with plasma exchange, haemodialysis and immunosuppressive drugs resulted in a rapid reduction in levels of immune complexes and other mediators of inflammation and was associated with good recovery of renal and lung function. Subsequently, deterioration in renal function occurred whilst the patient was on treatment with prednisolone alone but this was reversed with a short course of plasma exchange and the addition of azathioprine. No further deterioration in renal or lung function has been observed during 18 months treatment with azathioprine and prednisolone. Immediate plasma exchange and immunosuppressive drug treatment have been recommended for Goodpasture's syndrome. Immune complex mediated lung haemorrhage and nephritis is the main clinical differential diagnosis. Our case suggests that the same treatment is effective for both conditions if given early, and that detailed renal and immunological investigations should not be allowed to delay this.
一名16岁男孩患上了一种免疫复合物疾病,伴有肺出血、新月体增生性肾炎和肾衰竭。采用血浆置换、血液透析和免疫抑制药物治疗后,免疫复合物及其他炎症介质水平迅速下降,且肾功能和肺功能恢复良好。随后,患者在单独使用泼尼松龙治疗期间肾功能恶化,但通过短期血浆置换并加用硫唑嘌呤后得以逆转。在使用硫唑嘌呤和泼尼松龙治疗的18个月期间,未观察到肾功能或肺功能进一步恶化。对于肺出血肾炎综合征,建议立即进行血浆置换和免疫抑制药物治疗。免疫复合物介导的肺出血和肾炎是主要的临床鉴别诊断。我们的病例表明,如果早期给予相同治疗,对这两种病症均有效,且不应因详细的肾脏和免疫学检查而延误治疗。