Department of Nephrology, National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China.
Department of Radiology, National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China.
Semin Arthritis Rheum. 2022 Dec;57:152082. doi: 10.1016/j.semarthrit.2022.152082. Epub 2022 Aug 17.
The association of bronchiectasis with myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-AAV) has been widely described in recent studies. However, the clinical features and outcomes of MPO-ANCA-associated glomerulonephritis (MPO-ANCA GN) patients with bronchiectasis remain enigmatic.
MPO-ANCA GN patients with bronchiectasis were compared to MPO-ANCA GN patients alone. Clinical imaging, pathological tests, and follow-up examination data of patients were collected retrospectively. Progression to end-stage renal disease (ESRD) and death was treated as endpoint events.
153 cases (52 patients with bronchiectasis) were included in this study. Compared to MPO-ANCA GN patients alone, MPO-ANCA GN patients with bronchiectasis exhibited a lower level of proteinuria (p = 0.019) and relatively higher eGFR level. MPO-ANCA GN patients with bronchiectasis had less frequent incidences of interstitial lung disease (ILD) and emphysema (p<0.001, p = 0.016, respectively) but with higher rates of pulmonary infection (p<0.001). Bronchiectasis severity (the modified Reiff score) was positively correlated with MPO antibody titers (ρ=0.480, p<0.001), but not with shorter renal survival. A relatively higher remission rate was been seen in MPO-ANCA GN patients with bronchiectasis, who showed reduced susceptibility in progressing to ESRD in multivariate analysis (p = 0.043, HR=0.542, 95% CI 0.299-0.982). One-and three-year overall survival rates were 88.2% and 77.3% for MPO-ANCA GN with bronchiectasis cases versus 83.7% and 67.2% for MPO-ANCA GN patients alone (p = 0.431, p = 0.241, respectively).
The severity of bronchiectasis was correlated with anti-MPO antibody titers in MPO-ANCA GN patients. For MPO-ANCA GN patients, bronchiectasis associated with good renal prognosis, but it did not improve overall survival.
近年来的研究广泛描述了支气管扩张与髓过氧化物酶（MPO）抗中性粒细胞胞质抗体（ANCA）相关性血管炎（MPO-AAV）之间的关联。然而，伴有支气管扩张的 MPO-ANCA 相关性肾小球肾炎（MPO-ANCA GN）患者的临床特征和结局仍不清楚。
将伴有支气管扩张的 MPO-ANCA GN 患者与单纯 MPO-ANCA GN 患者进行比较。回顾性收集患者的临床影像学、病理检查和随访检查数据。将进展为终末期肾病（ESRD）和死亡作为终点事件。
本研究共纳入 153 例（52 例伴有支气管扩张）患者。与单纯 MPO-ANCA GN 患者相比，伴有支气管扩张的 MPO-ANCA GN 患者蛋白尿水平较低（p=0.019），eGFR 水平相对较高。伴有支气管扩张的 MPO-ANCA GN 患者发生间质性肺病（ILD）和肺气肿的频率较低（p<0.001，p=0.016），但肺部感染的发生率较高（p<0.001）。支气管扩张严重程度（改良 Reiff 评分）与 MPO 抗体滴度呈正相关（ρ=0.480，p<0.001），但与肾脏存活率无关。伴有支气管扩张的 MPO-ANCA GN 患者的缓解率相对较高，多因素分析显示其进展为 ESRD 的易感性降低（p=0.043，HR=0.542，95%CI 0.299-0.982）。伴有支气管扩张的 MPO-ANCA GN 患者 1 年和 3 年的总生存率分别为 88.2%和 77.3%，而单纯 MPO-ANCA GN 患者分别为 83.7%和 67.2%（p=0.431，p=0.241）。
在 MPO-ANCA GN 患者中，支气管扩张的严重程度与抗 MPO 抗体滴度相关。对于 MPO-ANCA GN 患者，支气管扩张与良好的肾脏预后相关，但不能改善总体生存率。
