Fujinami M, Sato K, Tateishi M, Okuda M, Akashi T, Okeda R, Miyasaka N
First Department of Internal Medicine, Tokyo Medical and Dental University.
Ryumachi. 1992 Apr;32(2):163-7; discussion 167-9.
An autopsy case of SLE died from acute and diffuse pulmonary hemorrhage is presented. A 50 year-old woman with SLE was admitted to our hospital because of high fever, butterfly rash, discoid skin lesions and renal dysfunction. She died from acute respiratory failure before initiation of the therapy with corticosteroid. Autopsy findings revealed a massive acute intrapulmonary hemorrhage. Histological study demonstrated a pulmonary arterial vasculitis with prominent fibrinoid necrosis at muscular pulmonary artery. No remarkable deposit of immunoglobulins and complements was found within the alveolar walls and pulmonary vessels by immunofluorescence and electron microscopy. Renal histology revealed diffuse proliferative glomerulonephritis with fibrinoid necrosis, crescent formation and wireloop lesions compatible with type IVb according to the WHO classification. The granular deposit of IgM, C3 and Clq, and electron dense deposit was found by immunofluorescence and by electron microscopy, respectively, in the kidney. The small arteries and veins in other organs, such as liver, spleen, bladder, ovary and rectum also revealed fibrinoid vasculitis. Acute infectious lesion was not observed in any tissue examined. The diffuse pulmonary hemorrhage in SLE could be one of the manifestations of active and severe systemic vasculitis.
本文报告一例死于急性弥漫性肺出血的系统性红斑狼疮(SLE)尸检病例。一名50岁的SLE女性因高热、蝶形红斑、盘状皮肤损害及肾功能不全入院。在开始使用糖皮质激素治疗前,她死于急性呼吸衰竭。尸检发现大量急性肺内出血。组织学研究显示肺动脉血管炎,肌性肺动脉有明显的纤维蛋白样坏死。免疫荧光和电子显微镜检查未在肺泡壁和肺血管内发现明显的免疫球蛋白和补体沉积。肾脏组织学显示弥漫性增殖性肾小球肾炎,伴有纤维蛋白样坏死、新月体形成及符合世界卫生组织(WHO)IVb型的线圈样病变。免疫荧光和电子显微镜检查分别在肾脏发现IgM、C3和Clq的颗粒状沉积以及电子致密沉积物。肝脏、脾脏、膀胱、卵巢和直肠等其他器官的小动脉和小静脉也显示纤维蛋白样血管炎。在所检查的任何组织中均未观察到急性感染性病变。SLE中的弥漫性肺出血可能是活动性严重系统性血管炎的表现之一。