显微镜下多血管炎肺部病变临床回顾性研究（附94例报告）

Clinical retrospective study on microscopic polyangiitis patients with lung involvement: 94 case reports attached

摘要:

目的：分析显微镜下多血管炎（microscopic polyangiitis，MPA）肺部病变特点及治疗方式，提高对MPA肺部受累的认识及诊疗水平。方法：回顾性分析2011年5月至2016年10月收治于重庆医科大学附属第一医院的94例MPA肺部受累患者的临床资料，包括临床表现、实验室检查、胸部影像学、病理学、治疗及预后等，并将上述患者分为以呼吸道症状为首发表现患者组（A组，34例）和以非呼吸道症状为首发表现患者组（B组，60例），比较2组患者的主要临床症状、实验室检查和胸部CT表现。结果：男49例，女45例，平均年龄（65.2±10.9）岁。呼吸系统以咳嗽、咳痰、咯血及呼吸困难为主要表现，呼吸衰竭者21例。胸部CT或高分辨CT表现主要分为以下3类：①以斑片或磨玻璃影为主要表现者61例（64.89%）；②以网格影或蜂窝样改变为主要表现者13例（13.83%）；③上述2种表现均存在者20例（21.28%）。12例（12.77%）影像学特点符合普通型间质性肺炎（usual interstitial pnermonia，UIP），30例（31.91%）符合弥漫性肺泡出血（diffuse alveolar hemorrhage，DAH）特点。A组呼吸系统表现发生率较B组高，差异有统计学意义（P＜0.05），如咳嗽/咳痰（32/34 vs. 46/60）、咯血（15/34 vs. 13/60）、呼吸困难（27/34 vs. 20/60），而全身症状及其他肺外表现2组无明显差异（P＞0.05），如发热（24/34 vs. 37/60）、皮疹（3/34 vs. 8/60）、血尿（22/34 vs. 41/60）、蛋白尿（21/34 vs. 40/60）等。A组胸部CT中DAH发生率较B组高（18/34 vs. 12/60），差异有统计学意义（P＜0.05）。82例接受治疗的患者均采用糖皮质激素和免疫抑制剂治疗，其中重症/危及生命患者中有11例采用甲泼尼龙+环磷酰胺静脉冲击治疗。结论：显微镜下多血管炎肺部受累发生率高，胸部影像学上以肺间质纤维化和DAH多见，DAH更易发生于以呼吸系统症状为首发表现的MPA患者。MPA治疗以激素联合免疫抑制剂为主，治疗短期预后较好。

Abstract:

Objective：To analyze the lung involvement characteristics of microscopic polyangiitis（MPA） and its treatments with the purpose of improving the awareness of it and the diagnosis and treatment for it. Methods：The clinical data of 94 MPA patients with lung involvement admitted into The First Affiliated Hospital of Chongqing Medical University from May 2011 to October 2016 were retrospectively analyzed，including the clinical manifestations，laboratory test results，chest imaging features，pathological features，treatment，prognosis etc. The main clinical manifestations，laboratory test results and chest imaging features were compared between patients with respiratory symptoms as initial symptoms（group A，n=34） and patients with non-respiratory symptoms as initial symp－toms（group B，n=60）. Results：The patients included 49 males and 45 females，with the mean age as （65.2±10.9） yeas. In MPA pa－tients with lung involvement，main manifestations were cough，expectoration，hemoptysis and dyspnea，and 21 patients were found with respiratory failure. The characteristics in chest CT/HRCT included the following patterns，patchy consolidation or ground-glass opaci－ties（n=61，64.89%），reticular or honeycombing（n=13，13.83%），and both of the former two（n=20，21.28%）. Twelve cases（12.77%） had radiographic evidences for usual interstitial pneumonia（UIP），and 30 cases（31.91%） cases for diffuse alveolar hemorrhage（DAH）. The incidence of respiratory symptoms in group A was significantly higher than that in group B（P<0.05），including cough/expectoration（32/34 vs. 46/60），hemoptysis（15/34 vs. 13/60） and dyspnea（27/34 vs. 20/60）. No significant differences were found between group A and B in constitutional symptoms and other extra-pulmonary manifestation（P>0.05），such as fever（24/34 vs. 37/60），rash（3/34 vs. 8/60），hematuresis（22/34 vs. 41/60），proteinuria （21/34 vs. 40/60）. The incidence of DAH in chest CT was sig－nificantly higher in group A than that in group B（18/34 vs. 12/60，P<0.05）. A total of 82 patients who accepted treatment were treated with glucocorticoids and immunosuppressive agents，among which，11 severe/life-threatening patients were treated with intravenous pulsed methylprednisolone with cyclophosphamide. Conclusion：The prevalence of lung involvement in patients with MPA is high，and pulmonary fibrosis and DAH are the special radiological findings. DAH is more likely to occur in patients with respiratory symptoms as initial symptoms. Glucocorticoids combined with immunosuppressive agents are the main treatment of MPA patients，whose short-term prognosis is relatively good.