Department of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Research Institute, 2-4-20 Ohgimachi, Kita-ku, Osaka, 530-8480, Japan.
Department of Nephrology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan.
BMC Nephrol. 2019 Dec 18;20(1):471. doi: 10.1186/s12882-019-1663-9.
Poststreptococcal acute glomerulonephritis (PSAGN) in the elderly tends to have a severe clinical course and often presents with crescentic necrotizing glomerulonephritis in the renal biopsy. However, vasculitis lesions are unusual.
We present a 71-year-old man who was admitted to our hospital for a recurrent gout attack with a rapid decline of renal function. Low C3 levels and a high anti-streptolysin O titer were observed, while myeloperoxidase- and proteinase 3- antineutrophil cytoplasmic antibody (ANCA) were negative. In addition to cellular crescent and necrosis lesions, diffuse peritubular capillaritis and venulitis as well as small arteriole vasculitis in the glomerular hilus were also apparent. Although granular C3c deposits in the capillary wall and hump lesions were not found, immunofluorescent staining for nephritis-associated plasmin receptor (NAPlr) and in situ zymography for plasmin activity were both positive. We thus diagnosed PSAGN accompanied by small vessel vasculitis. Steroid therapy gradually improved the patient's renal function, and hemodialysis was discontinued after 1 month.
In our case, streptococcus infection might have concurrently provoked vasculitis, and NAPlr staining was useful for confirming diagnosis.
老年人的链球菌后急性肾小球肾炎(PSAGN)往往具有严重的临床病程,且肾活检常表现为新月体性坏死性肾小球肾炎。然而,血管炎病变并不常见。
我们报告了一位 71 岁男性,因复发性痛风发作导致肾功能迅速下降而入院。观察到低 C3 水平和高抗链球菌溶血素 O 滴度,而髓过氧化物酶和蛋白酶 3-抗中性粒细胞胞质抗体(ANCA)为阴性。除细胞性新月体和坏死病变外,还明显存在弥漫性肾小管毛细血管炎和静脉炎以及肾小球门小动脉炎。虽然未发现毛细血管壁颗粒状 C3c 沉积和驼峰病变,但肾炎相关纤溶酶受体(NAPlr)免疫荧光染色和纤溶酶活性原位酶谱均为阳性。因此,我们诊断为伴有小血管血管炎的 PSAGN。类固醇治疗逐渐改善了患者的肾功能,1 个月后停止了血液透析。
在我们的病例中,链球菌感染可能同时引发了血管炎,NAPlr 染色有助于确诊。