ANCA相關性血管炎肺部臨床特征研究

蘇抗　陳希勝

摘要：目的 ?探討抗中性粒細胞胞漿抗體相關性小血管炎（AASV）患者肺臟受累的臨床特征，以提高對AASV的認識及診斷水平。方法 ?回顧性分析2015年3月～2019年9月于北京市順義區醫院確診為AASV的23例住院患者的臨床資料，分析其一般資料、臨床表現、實驗室檢查、肺部影像學檢查、病例活檢、預后等情況。結果 ?23例患者疾病首發癥狀方面，9例為呼吸系統癥狀、11例為泌尿系統癥狀、1例為神經系統癥狀、1例為消化系統癥狀、1例為骨關節癥狀;肺部表現主要為咳嗽（69.57%）、咳痰（65.22%）、胸悶（65.22%）、氣短（43.48%）、呼吸困難（34.78%）、咳血（21.74%）及肺間質病變（34.78%）等;實驗室檢查顯示均有C反應蛋白和血沉升高，大部分有中性粒細胞升高（69.57%）、貧血（65.22%），23例中抗中性粒細胞胞漿抗體（ANCA）均為核周型抗中性粒細胞胞漿抗體（p-ANCA）陽性（100.00%）;肺外表現主要為發熱（65.22%）、乏力（50.00%）、腎臟受累（95.65%）等;給予激素及免疫抑制劑治療后病情好轉20例（86.96%）;無效3例（13.04%），患者均死亡，1例因彌漫性肺泡出血死于失血性休克，1例因激素及免疫抑制劑使用后出現重癥感染死亡，1例因腎衰竭死亡。結論 ?AASV臨床表現無顯著特異性，大多具有多臟器、多系統受累的特點，其中以腎臟及肺臟累及最為多見，對于長期發熱同時合并多器官受損的患者應盡早進行ANCA 檢查，及早診斷，及時治療，以改善患者預后。

關鍵詞：ANCA 相關性血管炎;抗中性粒細胞胞漿抗體;肺部

中圖分類號：R593.2 ? ? ? ? ? ? ? ? ? ? ? ? ? ? ? ? ?文獻標識碼：A ? ? ? ? ? ? ? ? ? ? ? ? ? ? ? ?DOI：10.3969/j.issn.1006-1959.2020.18.036

文章編號：1006-1959（2020）18-0109-05

Study on the Clinical Characteristics of ANCA-associated Systemic Vasculitis

SU Kang，CHEN Xi-sheng

（Department of Respiratory Medicine，Shunyi District Hospital，Beijing 101300，China）

Abstract：Objective ?To explore the clinical features of lung involvement in patients with anti-neutrophil cytoplasmic antibody-associated small vasculitis （AASV） in order to improve the understanding and diagnosis of AASV.Methods ?The clinical data of 23 inpatients diagnosed with AASV in Shunyi District Hospital of Beijing from March 2015 to September 2019 were retrospectively analyzed， and their general data， clinical manifestations， laboratory examinations， lung imaging examinations， and cases were analyzed biopsy， prognosis， etc.Results ?In 23 patients， the first symptoms of the disease were respiratory symptoms in 9 cases， urinary system symptoms in 11 cases， neurological symptoms in 1 case， digestive system symptoms in 1 case， and bone and joint symptoms in 1 case; lung manifestations were mainly cough （69.57%）， expectoration （65.22%）， chest tightness （65.22%）， shortness of breath （43.48%）， dyspnea （34.78%）， hemoptysis （21.74%） and interstitial lung disease （34.78%）， etc.; laboratory examination showed that C-reactive protein and erythrocyte sedimentation rate were elevated， most of which had elevated neutrophils （69.57%） and anemia （65.22%）. Among the 23 cases， anti-neutrophil cytoplasmic antibodies （ANCA） were all pernuclear type antineutrophil cytoplasmic antibody （p-ANCA） positive （100.00%）; extrapulmonary manifestations are mainly fever （65.22%）， fatigue （50.00%）， kidney involvement （95.65%）， etc.; hormones and immunosuppression are given 20 patients （86.96%） improved after drug treatment; 3 patients （13.04%） were ineffective， all patients died， 1 patient died of hemorrhagic shock due to diffuse alveolar hemorrhage， and 1 patient developed severe infection after using hormones and immunosuppressants death， 1 case died of renal failure.Conclusion The clinical manifestations of AASV had no significant specificity， and most of them had the characteristics of multi-organ and multi-system involvement. Among them， kidney and lung involvement were the most common. For patients with long-term fever and multiple organ damage， ANCA examination should be performed as soon as possible for early diagnosis，timely treatment to improve the prognosis of patients.

Key words：ANCA-associated systemic vasculitis;Anti-neutrophil cytoplasmic antibody;Lung

抗中性粒細胞胞漿抗體（anti-neutrophil cytoplasmic antibody，ANCA）相關性小血管炎（ANCA-associated systemic vasculitis，AASV）是一種不常見的系統性自身免疫性疾病，主要特征是血清中存在髓過氧化物酶（MPO）靶抗原或蛋白酶3（PR3）靶抗原的相應MPO-ANCA或PR3-ANCA，病理特征為小血管壁炎癥和纖維素樣壞死。……