Association of Serum C3 Concentration and Histologic Signs of Thrombotic Microangiopathy with Outcomes among Patients with ANCA-Associated Renal Vasculitis

血清 C3 浓度和血栓性微血管病的组织学征象与 ANCA 相关性肾血管炎患者预后的关系

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作者:Lucio Manenti, Augusto Vaglio, Elisa Gnappi, Umberto Maggiore, Landino Allegri, Marco Allinovi, Maria L Urban, Marco Delsante, Maricla Galetti, Maria Nicastro, Francesco P Pilato, Carlo Buzio

Conclusions

Low sC3 levels and histologic signs of TMA are associated with a poor renal prognosis in patients with AAV.

Results

sC3 was below the lower limit of normal in 35% of the patients, whereas C4 was low in only 2%. Patients with low sC3 tended to be older (P=0.04) and to have lower eGFR at diagnosis (P=0.06). The median follow-up was 78 months (interquartile range, 18-135 months); 18 patients reached ESRD (10 of 14 and 8 of 26 in the low and normal sC3 groups, respectively). Death-censored renal survival was lower in the low sC3 group than in the normal sC3 group (log-rank test, P=0.01). Eight of the 30 patients who had undergone biopsy (27%) had histologic signs of TMA; these signs were more frequent in patients with low sC3 (5 of 10 versus 3 of 20; P=0.04). Notably, patients with histologic signs of TMA had a dramatically worse death-censored renal survival than patients without TMA (log-rank test, P=0.01), with ESRD occurring in 8 of 8 patients with TMA versus 8 of 22 patients without TMA. Conclusions: Low sC3 levels and histologic signs of TMA are associated with a poor renal prognosis in patients with AAV.

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