Orfila C, Meeus F, Bernadet P, Lepert J C, Suc J M
INSERM U 133, Toulouse-Rangueil, France.
Am J Nephrol. 1991;11(1):67-72. doi: 10.1159/000168276.
A 22-year-old woman presented glomerulonephritis with Schönlein-Henoch-like syndrome and monoclonal abnormality. One month later, she developed a rapidly progressive glomerulonephritis with hypertension and persistent purpura. In the two renal biopsies performed during the first and the second attack, mesangial expansion and thickening of the glomerular capillary walls (associated with 50% of crescents in the second biopsy) were observed on light microscopy. By immunofluorescence faint deposits of immunoglobulins (light and heavy chains) and complement components were found present in the mesangium. Electron microscopy showed tubular microfibrils measuring 19-24 nm in the mesangium, subendothelial and subepithelial areas. A skin biopsy performed during the first attack demonstrated leukocytoclastic skin vasculitis. By immunofluorescence, no deposits were observed. Congo red staining for amyloid and cryoglobulinemia were negative. This case is similar to an entity recently described and named immunotactoid glomerulopathy.
一名22岁女性出现伴有过敏性紫癜样综合征和单克隆异常的肾小球肾炎。1个月后，她发展为伴有高血压和持续性紫癜的快速进行性肾小球肾炎。在首次发作和第二次发作期间进行的两次肾活检中，光镜下观察到系膜扩张和肾小球毛细血管壁增厚（第二次活检中伴有50%的新月体形成）。免疫荧光检查发现系膜中有微弱的免疫球蛋白（轻链和重链）和补体成分沉积。电子显微镜显示系膜、内皮下和上皮下区域有直径为19 - 24 nm的管状微纤维。首次发作期间进行的皮肤活检显示白细胞破碎性皮肤血管炎。免疫荧光检查未观察到沉积物。淀粉样蛋白和冷球蛋白血症的刚果红染色均为阴性。该病例类似于最近描述并命名为免疫触须样肾小球病的一种疾病。
