Department of Nephrology, Tianjin Medical University General Hospital, Tianjin, 300052, China.
Department of Hematology, Tianjin Medical University General Hospital, Tianjin, 300052, China.
BMC Nephrol. 2019 Jul 30;20(1):287. doi: 10.1186/s12882-019-1487-7.
Many patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) need dialysis at disease onset due to severe kidney injury. Determining whether they can become dialysis independent is an important clinical assessment.
Forty kidney biopsy-proved myeloperoxidase (MPO)-ANCA associated AAV patients who required dialysis at disease onset were enrolled. Relationships between laboratory and pathological characteristics and prognoses were analyzed.
Twenty-five patients obtained dialysis independence within 3 months, while the other 15 patients remained dialysis dependent. No sclerotic class was identified among the 40 patients. Only two biopsies exhibited focal class diagnoses and both these patients recovered their renal function. The renal recovery rate of the 20 patients with mixed class was significantly lower than that of the 18 patients with crescentic class (40.0% vs. 83.3%, p = 0.006). Receiver operating characteristics (ROC) curves showed fibrous crescent+global glomerulosclerosis greater than 32.6% was a strong predictor of dialysis dependence with a sensitivity of 93.3% and specificity of 88.0%. When the percentage of fibrous crescent+global glomerulosclerosis exceeded 47.9%, dialysis independence was not possible. Correlation analysis indicated that platelet counts were negatively correlated with the percentage of fibrous crescent+global glomerulosclerosis (R = -0.448, p = 0.004). Most patients with increased platelets (84.62%) obtained renal recovery. Compared with methylprednisolone pulse therapy, plasma exchange accelerated renal recovery (29.4 ± 15.6 vs. 41.4 ± 11.7 days, p = 0.039).
For MPO-ANCA AAV who required dialysis at disease onset, crescentic and mixed classes accounted for the majority of patients in our cohort. The renal outcome of mixed class patients was worse than that of crescentic class. A high proportion of fibrous crescent+global glomerulosclerosis is a predictor of dialysis dependence. Increased platelet count is associated with active and reversible renal lesions.
许多抗中性粒细胞胞质抗体(ANCA)相关性血管炎(AAV)患者在疾病发作时因严重的肾脏损伤需要透析。确定他们是否能够摆脱透析是一个重要的临床评估。
本研究纳入了 40 名经肾活检证实为髓过氧化物酶(MPO)-ANCA 相关 AAV 且在疾病发作时需要透析的患者。分析了实验室和病理特征与预后之间的关系。
25 名患者在 3 个月内摆脱了透析,而另外 15 名患者仍依赖透析。40 名患者中没有发现硬化性病变。只有 2 例活检显示局灶性病变,这 2 例患者均恢复了肾功能。20 例混合性病变患者的肾脏恢复率明显低于 18 例新月体性病变患者(40.0%比 83.3%,p=0.006)。受试者工作特征(ROC)曲线显示,纤维性新月体+全球肾小球硬化大于 32.6%是透析依赖的强预测因素,其灵敏度为 93.3%,特异性为 88.0%。当纤维性新月体+全球肾小球硬化的百分比超过 47.9%时,就不可能摆脱透析。相关性分析表明,血小板计数与纤维性新月体+全球肾小球硬化的百分比呈负相关(R=-0.448,p=0.004)。大多数血小板升高的患者(84.62%)获得了肾脏恢复。与甲基强的松龙脉冲治疗相比,血浆置换加速了肾脏恢复(29.4±15.6 比 41.4±11.7 天,p=0.039)。
对于在疾病发作时需要透析的 MPO-ANCA AAV,新月体性和混合性病变在本队列中占大多数。混合性病变患者的肾脏预后较新月体性病变差。纤维性新月体+全球肾小球硬化比例高是透析依赖的预测因素。血小板计数升高与活动性和可逆转的肾脏病变有关。