强直性脊柱炎相关IgA肾病的临床及病理特点

摘要

Objective To investigate the clinical and pathologic characteristics of IgA nephropathy ( IgAN ) associated with ankylosing spondylitis ( AS ) . Methods The clinical and pathological data of 15 patients suffered from IgAN associated with AS in our hospital between 2009 and 2015 were reviewed. Results Eleven males and four females were included with the mean age of (35.7 ±9.2) years old. Fourteen patients (93.3%) presented HLA-B27 gene positive, eight patients revealed high serum IgA, four patients had hypertension.The renal manifestations showed insidious onset, ten cases presented with hematuria and proteinuria, five with asymptomatic hematuria, the average 24 hours proteinuria was (0.72 ±0.59 ) g, Serum creatinine levels of all the patients were normal.Renal biopsy pathology showed that the most common change was mild mesangial proliferation , eleven cases showed glomerulosclerosis, eight cases showed segmental sclerosis and thirteen cases with crescents formation, twelve cases showed interstitial vascular lesions.Conclusion The major clinical characteristics of IgAN associated with AS is hematuria and its main pathological changes included mild mesangial proliferation and crescents formation.%目的：分析强直性脊柱炎（ankylosing spondylitis， AS）相关IgA肾病（IgA nephropathy， IgAN）的临床病理特点。方法回顾性分析2009年1月至2015年1月在本院确诊为AS并发IgAN 患者的临床表现、病理特点、治疗方案及随访情况。结果 AS相关IgAN患者15例，肾活检时年龄（35.7±9.2）岁，男性11例，女性4例，14例（93.3％） HLA-B27阳性，8例可见血清IgA升高；4例高血压。肾损害多数起病隐匿，10例表现为蛋白尿并发血尿，5例以镜下血尿为主，24 h尿蛋白定量为（0.72±0.59） g，血肌酐水平均正常。肾病理以轻度系膜细胞增生为主，11例可见肾小球球性硬化，8例可见肾小球节段硬化，13例可见新月体形成，13例可见灶性肾间质纤维化，12例可见肾间质血管病变。结论 AS相关IgAN起病隐匿，容易漏诊。血尿是最突出的临床表现，轻度系膜增生和新月体形成是最常见的病理改变。通过早期诊断和较积极的免疫抑制剂治疗效果良好，预后尚可。