Norheim Katrine Brakke, Leh Sabine, Gøransson Lasse
Clinical Immunology Unit, Department of Internal Medicine, Stavanger University Hospital Stavanger, Norway.
Scand J Urol Nephrol. 2011 Dec;45(6):470-2. doi: 10.3109/00365599.2011.568955. Epub 2011 Oct 13.
This article reports a case of Goodpasture's syndrome and microangiopathic haemolytic anaemia developing in a patient with a 2-year history of systemic sclerosis. Goodpasture's syndrome usually presents with lung haemorrhage and acute renal failure, and kidney biopsy typically shows crescentic glomerulonephritis with linear immunoglobulin staining on the glomerular basement membrane. Treatment of choice for Goodpasture's syndrome is immunosuppressive therapy and plasma exchange, which has greatly improved morbidity and mortality. This unusual case highlights that seemingly unrelated autoimmune diseases may coexist in one patient, and thorough clinical examination combined with serology and histology may be necessary for correct diagnosis.
本文报道了一例患有2年系统性硬化症病史的患者发生古德帕斯彻综合征和微血管病性溶血性贫血的病例。古德帕斯彻综合征通常表现为肺出血和急性肾衰竭，肾脏活检典型表现为新月体性肾小球肾炎，肾小球基底膜有线性免疫球蛋白染色。古德帕斯彻综合征的首选治疗方法是免疫抑制治疗和血浆置换，这已大大改善了发病率和死亡率。这个不寻常的病例突出表明，看似不相关的自身免疫性疾病可能在同一患者中共存，正确诊断可能需要全面的临床检查并结合血清学和组织学检查。
