Kikuchi Y, Yoshizawa N, Oda T, Imakiire T, Suzuki S, Miura S
Second Department of Internal Medicine, National Defense Medical College, Namiki, Tokorozawa, Saitama, Japan.
Clin Nephrol. 2006 Feb;65(2):124-8. doi: 10.5414/cnp65124.
We report the case of a 25-year-old man with abdominal pain, purpura on the legs and proteinuria occurring 2 weeks after acute tonsillitis, and admitted to our hospital with suspected Henoch-Schönlein purpura nephritis (HSPN). He had increased anti-streptolysin O (ASO) titer and hypocomplementemia. A renal biopsy specimen showed endocapillary proliferative changes, which are typical of acute poststreptococcal glomerulonephritis (APSGN). However, immunofluorescence study revealed predominant IgA and C3 deposits in mesangial lesions, indicating a diagnosis of HSPN. Because of massive proteinuria initially, the treatment with a combination of prednisolone, cyclophosphamide, dipyridamole and warfarin was started along with 3 plasma exchanges. Angiotensin-converting enzyme inhibitor was also given. Response to the treatment was favorable. A follow-up biopsy was performed 8 months after the first biopsy. The renal biopsy specimen showed a figure of typical HSPN. To further investigate the cause of glomerular changes in our patient, an immunofluorescent study of nephritogenic nephritis-associated plasmin receptor (NAPlr) of group A, beta-hemolytic streptococci was performed. NAPlr was significantly positive in the glomeruli in the first biopsy specimen, but not in the second. His clinical course and pathological findings suggest that NAPlr may be related to the pathogenesis in a part of patients with HSPN, especially in patients with high ASO titer and hypocomplementemia.
我们报告了一例25岁男性患者,在急性扁桃体炎发作2周后出现腹痛、腿部紫癜和蛋白尿,因疑似过敏性紫癜性肾炎(HSPN)入院。他的抗链球菌溶血素O(ASO)滴度升高且补体水平降低。肾活检标本显示毛细血管内增生性改变,这是急性链球菌感染后肾小球肾炎(APSGN)的典型表现。然而,免疫荧光研究显示系膜病变中主要为IgA和C3沉积,提示诊断为HSPN。由于最初存在大量蛋白尿,开始使用泼尼松龙、环磷酰胺、双嘧达莫和华法林联合治疗,并进行了3次血浆置换。同时给予血管紧张素转换酶抑制剂。治疗反应良好。首次活检8个月后进行了随访活检。肾活检标本显示为典型的HSPN表现。为进一步探究该患者肾小球病变的原因,对A组β溶血性链球菌的致肾炎性肾炎相关纤溶酶受体(NAPlr)进行了免疫荧光研究。在首次活检标本的肾小球中,NAPlr显著阳性,但在第二次活检中则为阴性。他的临床病程和病理结果提示,NAPlr可能与部分HSPN患者的发病机制有关,尤其是ASO滴度高且补体水平降低的患者。