Chen Z, Yang Y J, Li J, Tian X P
Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China.
Zhonghua Nei Ke Za Zhi. 2018 Feb 1;57(2):129-133. doi: 10.3760/cma.j.issn.0578-1426.2018.02.009.
To investigate the clinical features of Takayasu's arteritis (TAK) with glomerulonephropathy and to improve physicians' understanding of this complication in patients with TAK. Clinical data were retrospectively collected including manifestations, laboratory tests, image findings and treatment of 8 patients diagnosed as Takayasu's arteritis with glomerulonephropathy from January 2002 to January 2017 in Peking Union Medical College Hospital. Glomerulonephropathy was confirmed based on percutaneous renal biopsy. There were 6 women and 2 men. The median onset age and median disease duration were 24 (18-37) years and 42 (3-360) months, respectively. Five patients had hypertension. The 24 hour urinary protein was 0.18-14.91 g. Red blood cells and casts in urine were tested among 4 and 2 patients, respectively. Three patients had renal artery stenosis. Three patients demonstrated mesangial proliferative glomerulonephritis, two with IgA nephropathy, two with minimal change disease and one with membranoproliferative glomerulonephritis. Seven patients received glucocorticoid combined with cyclophosphamide therapy (glucocorticoid 40-60 mg/d, prednisone or equivalent; cyclophosphamide 0.4 g/week iv. or cyclophosphamide 0.1 g/d po.). Uninary blood cells removed and 24 hour urinary protein decreased from 1.65 g to 0.90 g after treatment for 12 months in one patient. The other 7 patients were missing. Glomerulonephropathy is occasionally observed among TAK patients. Mesangial proliferative glomerulonephritis is the most common pathological subtype. Glucocorticoid combined with cyclophosphamide therapy could be an optional therapy for Takayasu's arteritis with glomerulonephropathy.
探讨合并肾小球肾炎的大动脉炎(TAK)的临床特征,以提高医生对TAK患者这一并发症的认识。回顾性收集2002年1月至2017年1月在北京协和医院确诊为合并肾小球肾炎的大动脉炎患者的临床资料,包括临床表现、实验室检查、影像学检查及治疗情况。肾小球肾炎经皮肾穿刺活检确诊。患者中女性6例,男性2例。发病年龄中位数和病程中位数分别为24(18 - 37)岁和42(3 - 360)个月。5例患者有高血压。24小时尿蛋白为0.18 - 14.91g。4例患者尿红细胞检查阳性,2例患者尿中出现管型。3例患者有肾动脉狭窄。3例患者表现为系膜增生性肾小球肾炎,2例为IgA肾病,2例为微小病变型肾病,1例为膜增生性肾小球肾炎。7例患者接受糖皮质激素联合环磷酰胺治疗(糖皮质激素40 - 60mg/d,泼尼松或等效药物;环磷酰胺0.4g/周静脉注射或环磷酰胺0.1g/d口服)。1例患者治疗12个月后尿血细胞消失,24小时尿蛋白从1.65g降至0.90g。其他7例患者失访。TAK患者中偶尔会观察到肾小球肾炎。系膜增生性肾小球肾炎是最常见的病理亚型。糖皮质激素联合环磷酰胺治疗可能是合并肾小球肾炎的大动脉炎的一种可选治疗方法。