血栓性微血管病相关肾脏疾病患者的临床病理特点及预后

Clinico-pathological characteristics and prognosis in patients with thrombotic microangiopathy

目的：比较血栓性微血管病（TMA）相关肾脏疾病的临床、病理特征及预后。方法：选取肾活检病理为TMA病变的患者,选其中非典型溶血尿毒综合征（a HUS）38例、狼疮性肾炎相关性TMA（LN-TMA）37例、妊娠相关性TMA（P-TMA）22例,比较三组患者的临床、病理及预后。结果：a HUS组患者临床表现最重,血清肌酐水平[a HUS 791.2μmol/L（396.9～1 148.3μmol/L）vs LN-TMA 388.1μmol/L（189.2～581.7μmol/L）vs P-TMA 69.8μmol/L（53.9～556.9μmol/L）]及贫血的发生率（a HUS 100%vs LN-TMA 97.3%vs P-TMA 63.6%）均高于其他两组,肾脏病理TMA病变表现多样。P-TMA组病情最轻,肾功能不全的发生率（a HUS 100%vs LN-TMA 89.2%vs PTMA 50.0%）、血小板减少的发生率（a HUS 76.3%vs LN-TMA 75.7%vs P-TMA 27.3%）及贫血的发生率均显著低于其他两组,病理以肾小球基膜双轨病变（a HUS 55.3%vs LN-TMA 45.9%vs P-TMA 95.5%）最为突出。LN-TMA组蛋白尿水平[a HUS 1.38 g/24h（0.74～2.58 g/24h）vs LN-TMA 2.65 g/24h（1.87～5.33 g/24h）vs P-TMA 1.18g/24h（0.68～1.64 g/24h）]及镜下血尿的发生率（a HUS 65.8%vs LN-TMA 89.2%vs P-TMA 50.0%）最高,病理上动脉血栓（a HUS 57.9%vs LN-TMA 83.8%vs P-TMA 22.7%）及肾小球袢腔内血栓（a HUS 26.3%vs LN-TMA 59.5%vs P-TMA 4.5%）均位列三组之首。随访末a HUS组预后最差,P-TMA组预后最好（ESRD发生率a HUS 63.2%vs LNTMA 24.3%vs P-TMA 18.2%,P〈0.01）,LN-TMA组肾功能完全恢复比例最高（a HUS 15.8%vs LN-TMA 27.0%vs P-TMA 18.2%）。肾小管间质慢性病变与肾脏长期预后独立相关,动脉内膜纤维性增生、球性硬化及肾小管间质慢性病变比例高是影响肾功能不全逆转的危险因素。结论：TMA相关肾脏疾病临床特点、肾脏病理改变及预后存在显著差异,三组患者中a HUS组预后最差,LN-TMA组及P-TMA组预后相对较好。

Objective： To compare the clinico-pathological characteristics and prognosis in patients with different types of TMA by proven renal biopsy. Methodology： Ninty seven patients who diagnozed as TMA by renal biopsy were enrolled into this retrospective study. They included atypical hemolytic uremic syndrome（ a HUS）（ n = 38）,lupus nephritis-associated TMA（ LN-TMA）（ n = 37）,and pregnancy-associated TMA（ P-TMA）（ n = 22）. Their clinicopathological characteristics and the prognoses were compared. Results： The patients with a HUS presented a highest level of serum creatinine 791（ 397 ～ 1 148） μmol / L vs LN-TMA 388（ 189 ～ 582） μmol / L vs P-TMA 69. 8（ 53. 9 ～ 557）μmol /L）,and a highest incidence of anemia（ 100% vs LN-TMA 97. 3% vs P-TMA 63. 6%）,with a wide variety of TMA lesions. The patients with P-TMA presented the lowest level of systemic involvement,the lowest incidences of renal insufficiency（ a HUS 100% vs LN-TMA 89. 2% vs P-TMA 50. 0%）,anemia and thrombocytopenia（ a HUS 76. 3% vs LNTMA 75. 7% vs P-TMA 27. 3%）,and the highest incidence glomerular basement membrane（ GBM） reduplication（ a HUS55. 3% vs LN-TMA 45. 9% vs P-TMA 95. 5%）. However,the patients with LN-TMA presented the highest level of proteinuria（ a HUS 1. 38（ 0. 74 ～ 2. 58） g / 24 h vs LN-TMA 2. 65（ 1. 87 ～ 5. 33） g / 24 h vs P-TMA 1. 18（ 0. 68 ～ 1. 64）g / 24h）,the highest incidence of microscopic hematuria（ a HUS 65. 8% vs LN-TMA 89. 2% vs P-TMA 50. 0%）,and arterial and glomerular thrombi dominantly（ a HUS 57. 9% vs LN-TMA 83. 8% vs P-TMA 22. 7%; a HUS 26. 3% vs LNTMA 59. 5% vs P-TMA 4. 5%,respectively）. At the end of follow-up,a HUS patients showed the poorest renal outcomes to end-stage renal disease（ a HUS 63. 2% vs LN-TMA 24. 3% vs P-TMA 18. 2%,P〈0. 001）,whereas LN-TMA patients had the highest incidence of recovery of renal insufficiency（ a HUS 15. 8% vs LN-TMA 27. 0% vs P-TMA 18. 2%,P〈0. 001）.The percentage of tubular atrophy / interstitial fibrosis（ TAIF） independently influenced the renal survival. The presence of arteriolar / arterial intimal fibroplasia,the increased percentages of glomerulosclerosis and TAIF were risk factors for nonrecovering from renal insufficiency. Conclusion： The patients with different types of TMA presented a wide variety of clinico-pathological characteristics and prognoses. In these three groups,patients with a HUS had the worst prognoses,whereas patients with LN-TMA and P-TMA had better prognoses.