The spectrum of renal thrombotic microangiopathy in lupus nephritis.
The spectrum of renal thrombotic microangiopathy in lupus nephritis.
复制标题
狼疮性肾炎肾血栓性微血管病谱
DOI：
10.1186/ar4142
复制
发表时间：
2013-01-15
影响因子：
4.9
通讯作者：
Zhao MH
中科院分区：
文献类型：
--
作者：
Song D;Wu LH;Wang FM;Yang XW;Zhu D;Chen M;Yu F;Liu G;Zhao MH
IntroductionAmong various lupus renal vascular changes, thrombotic microangiopathy (TMA) presented with the most severe clinical manifestations and high mortality. The pathogenesis of TMA in systemic lupus erythematosus (SLE) was complicated. The aim of this study was to assess clinical manifestations, laboratory characteristics, pathological features and risk factors for clinical outcomes of lupus nephritis patients co-existing with renal TMA in a large cohort in China.MethodsClinical and renal histopathological data of 148 patients with biopsy-proven lupus nephritis were retrospectively analyzed. Serum complement factor H, A Disintegrin and Metalloprotease with Thrombospondin type I repeats 13 (ADAMTS-13) activity, antiphospholipid antibodies and C4d deposition on renal vessels were further detected and analyzed.ResultsIn the 148 patients with lupus nephritis, 36 patients were diagnosed as co-existing with renal TMA based on pathological diagnosis. Among the 36 TMA patients, their clinical diagnoses of renal TMA were as followings: 2 patients combining with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome, 2 patients combining with anti-phospholipid syndrome, 2 patients with malignant hypertension, 1 patient with scleroderma and the other 29 patients presenting with isolated renal TMA. Compared with the non-renal TMA group, patients with renal TMA had significantly higher urine protein (7.09 ± 4.64 vs. 4.75 ± 3.13 g/24h,P= 0.007) and serum creatinine (159, 86 to 215 vs. 81, 68 to 112 μmol/l,P<0.001), higher scores of total activity indices (AI) (P<0.001), endocapillary hypercellularity (P<0.001), subendothelial hyaline deposits (P= 0.003), interstitial inflammation (P= 0.005), glomerular leukocyte infiltration (P= 0.006), total chronicity indices (CI) (P= 0.033), tubular atrophy (P= 0.004) and interstitial fibrosis (P= 0.018). Patients with renal TMA presented with poorer renal outcome (P= 0.005) compared with the non-TMA group. Renal TMA (hazard ratio (HR): 2.772, 95% confidence interval: 1.009 to 7.617,P= 0.048) was an independent risk factor for renal outcome in patients with lupus nephritis. The renal outcome was poorer for those with both C4d deposition and decreased serum complement factor H in the TMA group (P= 0.007).ConclusionsThere were various causes of renal TMA in lupus nephritis. Complement over-activation via both classical and alternative pathways might play an important role in the pathogenesis of renal TMA in lupus nephritis.
登录
查看更多内容
影响因子：
5.5
作者：
Galindo, Maria;Gonzalo, Elena;Pablos, Jose L.
通讯作者：
Pablos, Jose L.
影响因子：
3.6
作者：
Lee, Beom Hee;Kwak, Soo Heon;Cheong, Hae Il
通讯作者：
Cheong, Hae Il
影响因子：
2.6
作者：
Li, S-J;Liu, Z-H;Li, L-S
通讯作者：
Li, L-S
影响因子：
13.2
作者：
BANFI, G;BERTANI, T;SACCHI, G
通讯作者：
SACCHI, G
影响因子：
5
作者：
Liu, Chau-Ching;Ahearn, Joseph M;Manzi, Susan
通讯作者：
Manzi, Susan