Department of Nephrology, General University Hospital and First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic.
Department of Pathology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.
Nephrol Dial Transplant. 2014 Sep;29(9):1728-32. doi: 10.1093/ndt/gfu042. Epub 2014 Feb 26.
Histopathological lesions in renal biopsy (RB) at presentation of ANCA-associated vasculitis (AAV) have been described in depth but repeat protocolized renal biopsies are seldomly performed in AAV. In this study, we present a group of AAV patients with repeat protocolized biopsies, and we evaluate their clinical significance.
A total of 17 consecutive patients diagnosed between 1991 and 1995 with AAV and renal involvement confirmed by biopsy at presentation in a single center underwent a protocol planned rebiopsy in remission after a median of 13 months (range 11-28) from diagnosis. Biopsies were assessed by two independent pathologists, blinded to patient data. Clinical data were collected retrospectively.
Patients were followed-up for a median of 189 months from diagnosis. Renal relapse was observed in eight patients (47.1%), seven patients died, three patients reached end-stage renal failure. There was a significant decrease in the percentage of acute lesions (cellular crescents, fibrinoid necrosis, P < 0.001) and a significant increase in chronic lesions (glomerulosclerosis, interstitial fibrosis, P ≤ 0.01) in the repeat RB compared with the first RB. This resulted in a class change over the biopsies within most patients. The percentage of normal glomeruli in the first biopsy positively correlated with estimated GFR at the end of follow-up (rs = 0.509, P = 0.05).
This is the first study on protocolized repeat biopsies in AAV, giving insight into disease activity under immunosuppressive treatment. Apparently, many AAV patients have grumbling disease with ongoing activity, eventually leading to an increased amount of chronic lesions.
在抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的肾活检(RB)中,已经对组织病理学病变进行了深入描述,但在 AAV 中很少重复进行协议化的肾活检。在这项研究中,我们报告了一组接受重复协议化活检的 AAV 患者,并评估了其临床意义。
1991 年至 1995 年间,在一家单中心诊断出 17 例连续的 AAV 患者,他们的肾受累在初次就诊时通过活检证实,中位随访时间为 13 个月(范围为 11-28),患者处于缓解期,进行了计划的重复肾活检。两名独立的病理学家对活检进行评估,不了解患者的数据。回顾性收集临床数据。
患者从诊断开始的中位随访时间为 189 个月。8 例(47.1%)患者出现肾复发,7 例患者死亡,3 例患者进入终末期肾衰竭。与初次 RB 相比,重复 RB 中急性病变(细胞性新月体、纤维蛋白样坏死,P<0.001)的比例显著下降,而慢性病变(肾小球硬化、间质纤维化,P≤0.01)的比例显著增加。在大多数患者中,活检结果的病变类型发生了变化。初次活检中正常肾小球的百分比与随访结束时的估计肾小球滤过率呈正相关(rs=0.509,P=0.05)。
这是第一项关于 AAV 重复协议化活检的研究,深入了解了免疫抑制治疗下的疾病活动情况。显然,许多 AAV 患者的疾病仍在活动中,最终导致慢性病变增多。