Jankovic Aleksandar, Maslarevic-Radovic Vesna, Djuric Petar, Tosic-Dragovic Jelena, Bulatovic Ana, Simovic Nikola, Mitrovic Milos, Stankovic-Popovic Verica, Dopudja-Pantic Vesna, Arandjelovic Snezana, Dimkovic Nada
Clinical Department for Nephrology with Dialysis, University Medical Center Zvezdara , Belgrade , Serbia.
Clinical Department for Pulmonology, University Medical Center Zvezdara , Belgrade , Serbia.
Front Immunol. 2017 Feb 13;8:111. doi: 10.3389/fimmu.2017.00111. eCollection 2017.
Microscopic polyangiitis (MPA) is one of the causes of the pulmonary-renal syndrome associated with elevated non-specific markers of inflammation and antineutrophil cytoplasmic autoantibody (ANCA) positivity in 50-75%. occurrence of the disease in patients on chronic hemodialysis (HD) has not been described.
We presented patient who developed MPO-ANCA-associated MPA with lung and musculoskeletal involvement after 4 years on regular HD due to bilateral nephrectomy. After excluding the other causes of MPO-ANCA positivity, diagnosis was confirmed even without renal biopsy. Patient received standard immunosuppression therapy and he is still in remission after 27 months.
The onset of immune-mediated disease could be observed even after introduction of renal replacement therapy, which may be a diagnostic problem. Early recognition and traditional immunosuppressive regiment may provide successful outcome.
显微镜下多血管炎(MPA)是肺肾综合征的病因之一,与炎症非特异性标志物升高以及50% - 75%的抗中性粒细胞胞浆自身抗体(ANCA)阳性有关。尚未有关于慢性血液透析(HD)患者发生该病的描述。
我们报告了一名患者,该患者在因双侧肾切除接受规律血液透析4年后发生了伴有肺部和肌肉骨骼受累的MPO - ANCA相关性MPA。在排除MPO - ANCA阳性的其他病因后,即使未进行肾活检也确诊了该疾病。患者接受了标准免疫抑制治疗,27个月后仍处于缓解期。
即使在引入肾脏替代治疗后仍可观察到免疫介导疾病的发作,这可能是一个诊断难题。早期识别和传统免疫抑制方案可能带来成功的治疗结果。