Department of Nephrology and Clinical Immunology, RWTH Aachen University Hospital, Aachen, Germany.
Department of Diagnostic and Interventional Radiology, Medical Faculty, University Dusseldorf, Düsseldorf, Germany.
Rheumatol Int. 2024 Nov;44(11):2435-2443. doi: 10.1007/s00296-024-05664-8. Epub 2024 Aug 13.
ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease.
抗中性粒细胞胞浆抗体相关性血管炎（AAV）包括肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎。所有形式都可能涉及不同的器官系统，但肾脏和肺部的受累在许多情况下是常见且致命的。在这里，我们旨在确定肺部疾病表现和个别 CT 发现在 AAV 患者中的预测价值。我们回顾性地分析了德国一家三级风湿病中心的 CT 扫描和临床资料，包括死亡率、肾脏结局、复发发生和损伤评分。我们共纳入了 94 名 AAV 患者（GPA 患者 49 名，MPA 患者 41 名）。44 名患者有肺部受累，且有可用的 CT 扫描，其中 70.5%的患者为 GPA，72.7%的患者有肾脏受累。结节形成和空洞在 GPA 患者中更为常见，而磨玻璃影（GGO）、间质性肺病和胸腔积液主要见于 MPA 患者。在中位随访 37 个月期间，GPA 患者的总体死亡率略高，而 MPA 患者的终末期肾衰竭发生率显著增加。两种疾病的复发频率相当。GGO 和胸腔积液的存在与更高的复发率相关，而结节与复发呈负相关。值得注意的是，与接受其他治疗的患者相比，接受 RTX 治疗的患者感染较少。我们的数据表明，特征性 CT 模式在 AAV 诊断评估中具有重要意义。特别是包括 GGO 和胸腔积液在内的某些 CT 模式可能有助于识别复发风险较高的患者。
