Antiphospholipid syndrome (APS) nephropathy in catastrophic, primary, and systemic lupus erythematosus-related APS.
Antiphospholipid syndrome (APS) nephropathy in catastrophic, primary, and systemic lupus erythematosus-related APS.
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发表时间：
2008-10
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通讯作者：
M. Tektonidou;F. Sotsiou;H. Moutsopoulos
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作者：
M. Tektonidou;F. Sotsiou;H. Moutsopoulos
目的 抗磷脂综合征 (APS) 中的肾脏受累尚未得到充分认识。最近在小范围的原发性 APS (PAPS) 和系统性红斑狼疮 (SLE)-APS 患者中观察到肾小血管病变，定义为 APS 肾病。我们检查了不同 APS 患者组（包括灾难性 APS (CAPS)）患者的肾脏组织学、临床和实验室特征。方法 我们的研究包括所有转诊至我们科室且活检证实有肾脏受累的 CAPS (n=6)、PAPS (n=8) 和 SLE-APS (n=23) 患者。肾活检标本由同一位肾脏病理学家进行回顾性检查。 APS 肾病的诊断如前所述。记录人口统计、临床和实验室数据。结果所有CAPS患者均存在符合APS肾病诊断的急性和慢性肾血管病变。在所有 CAPS 患者中均观察到血栓性微血管病 (TMA)，即急性病变。小叶间动脉纤维性内膜增生 (FIH) 和局灶性皮质萎缩 (FCA) 是最常见的慢性血管病变，分别发生在 6 名 CAPS 患者中的 4 名 (66.7%) 和 6 名 CAPS 患者中的 3 名 (50%)。 8 名 PAPS 患者中的 3 名 (37.5%) 和 23 名 SLE-APS 患者中的 8 名 (35%) 检测到 TMA，而 FIH 和 FCA 在所有 3 组中的频率相似。高血压、蛋白尿、血尿和肾功能不全是所有 APS 组最常见的肾脏表现。结论 3个APS组均检出急性和慢性APS肾病病变。急性病变在 CAPS 中更为突出，而慢性病变在所有组中的发生率相似。高血压、蛋白尿、血尿和肾功能不全是所有 APS 组最常见的肾脏表现。
OBJECTIVE Renal involvement in antiphospholipid syndrome (APS) has been poorly recognized. A renal small-vessel vasculopathy, defined as APS nephropathy, has recently been observed in small series of patients with primary APS (PAPS) and systemic lupus erythematosus (SLE)-APS. We examined the renal histologic, clinical, and laboratory characteristics of different groups of patients with APS including catastrophic APS (CAPS). METHODS Our study included all CAPS (n=6), PAPS (n=8), and SLE-APS (n=23) patients with biopsy-proven renal involvement who were referred to our departments. The kidney biopsy specimens were retrospectively examined by the same renal pathologist. APS nephropathy was diagnosed as previously described. Demographic, clinical, and laboratory data were recorded. RESULTS All patients with CAPS had acute and chronic renal vascular lesions compatible with diagnosis of APS nephropathy. Thrombotic microangiopathy (TMA), the acute lesion, was observed in all CAPS patients. Fibrous intimal hyperplasia of interlobular arteries (FIH) and focal cortical atrophy (FCA) were the most common chronic vascular lesions, occurring in 4 of 6 (66.7%) and 3 of 6 (50%) patients with CAPS, respectively. TMA was detected in 3 of 8 (37.5%) patients with PAPS and in 8 of 23 (35%) patients with SLE-APS, while FIH and FCA were found with similar frequencies in all 3 groups. Hypertension, proteinuria, hematuria, and renal insufficiency were the most common renal manifestations of all APS groups. CONCLUSION Acute and chronic APS nephropathy lesions were detected in all 3 APS groups. Acute lesions were more prominent in CAPS, while chronic lesions were found with similar frequencies in all groups. Hypertension, proteinuria, hematuria, and renal insufficiency were the most common renal manifestations of all APS groups.