Nakahara C, Hayashi D, Kinugasa H, Horigome H, Matsui A, Takagi A, Kato N, Kobayashi M
Department of Pediatrics, Tsukuba University Hospital, University of Tsukuba, Japan.
Clin Nephrol. 2001 Oct;56(4):332-5.
We report here on an 11-year-old Japanese girl who was found to have proteinuria by routine mass screening urinalysis for school children, and who developed systemic lupus erythematosus (SLE) 21 months later. The initial renal biopsy, performed 3 months after the first visit to Tokyo Medical University Kasumigaura Hospital (TMUKH), revealed membranous glomerulonephritis. In an immunofluorescent study, IgG was the only positive immunoglobulin found. A "full-house" immunofluorescence glomerulopathy, well known as a predictive finding for lupus nephritis, was not detected. Endothelial tubuloreticular inclusions (ETI) were found by electron microscopy. Because the diagnosis of SLE was not established clinically and serologically, the patient was followed every 3 months without drugs. Her urinary findings returned to normal within 18 months. Three months after the last visit, she was sent to Tsukuba University Hospital (TUH) for fever, arthralgia, dyspnea and butterfly rash. She was diagnosed as having SLE, pleuritis, and pericarditis. Although she was treated with methylpredonisolone and oral prednisolone, she developed cardiac tamponade on the 12th day of admission during the course of pneumococcal septicemia. Finally, she was treated successfully with surgical procedures, antibiotics and oral prednisolone and was discharged. We conclude that ETI is a more significant early sign of SLE than "full-house" immmunofluorescence glomerulopathy, especially in pediatric cases.
我们在此报告一名11岁的日本女孩,她在学校儿童常规尿液筛查中被发现蛋白尿,并在21个月后发展为系统性红斑狼疮(SLE)。首次就诊于东京医科大学霞浦医院(TMUKH)3个月后进行的初次肾活检显示为膜性肾小球肾炎。在免疫荧光研究中,IgG是唯一发现的阳性免疫球蛋白。未检测到众所周知的狼疮性肾炎预测性表现“满堂亮”免疫荧光肾小球病。通过电子显微镜发现了内皮细胞管型包涵体(ETI)。由于临床和血清学上未确诊SLE,该患者每3个月随访一次,未用药。她的尿液检查结果在18个月内恢复正常。最后一次就诊3个月后,她因发热、关节痛、呼吸困难和蝶形皮疹被送往筑波大学医院(TUH)。她被诊断为患有SLE、胸膜炎和心包炎。尽管她接受了甲泼尼龙和口服泼尼松龙治疗,但在入院第12天肺炎球菌败血症病程中出现了心脏压塞。最后,她通过手术、抗生素和口服泼尼松龙成功治疗并出院。我们得出结论,ETI是SLE比“满堂亮”免疫荧光肾小球病更重要的早期征象,尤其是在儿科病例中。