Feng Rui-e, Xu Wen-bing, Shi Ju-hong, Mahmoudi Artin, Mu Wen-bing, Zheng Wen-jie, Zhu Yuan-jue, Liu Hong-rui
Department of Pathology, Peking Union Medical College, Beijing 100730, China.
Chin Med Sci J. 2011 Mar;26(1):1-8. doi: 10.1016/s1001-9294(11)60012-6.
To investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis.
We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review. All patients underwent transbronchial lung biopsy (TBLB). Six of them underwent surgical lung biopsy as well.
The patients included 7 men and 9 women, aged from 14 to 61 years (median, 47.5 years). Extrathoracic organs involved included nervous system (7/16) and skin (5/16). Respiratory symptoms included cough (12/16), exertional dyspnea (11/16), hemoptysis (4/16), and chest pain (3/16). CT findings included bilateral ground-glass opacities (12/16), bilateral patchy opacities (12/16), and centrilobular nodules (6/16). The pathological findings of TBLB demonstrated increased eosinophils (3/16), vasculitis (3/16), and interstitial pneumonia (16/16). The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period (range, 3 to 51 months; median, 15 months).
Asthma may be present in CSS patient when there is bronchial involvement. Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.
研究变应性肉芽肿性血管炎（CSS）相关的肺部受累情况，重点关注临床特征、肺部受累的病理表现、治疗反应及预后。
我们回顾性分析了CSS的临床表现、薄层CT及病理特征。该研究纳入16例患者。通过查阅病历获取临床资料。所有患者均接受了经支气管肺活检（TBLB）。其中6例还接受了外科肺活检。
患者包括7名男性和9名女性，年龄14至61岁（中位数47.5岁）。胸外器官受累包括神经系统（7/16）和皮肤（5/16）。呼吸系统症状包括咳嗽（12/16）、劳力性呼吸困难（11/16）、咯血（4/16）和胸痛（3/16）。CT表现包括双侧磨玻璃影（12/16）、双侧斑片状影（12/16）和小叶中心结节（6/16）。TBLB的病理表现显示嗜酸性粒细胞增多（3/16）、血管炎（3/16）和间质性肺炎（16/16）。6例外科肺活检的病理表现显示4例有坏死性血管炎，5例有毛细血管病变，3例有嗜酸性肺炎，2例有肉芽肿，3例有气道异常。研究期间所有患者治疗后症状均有改善（范围3至51个月；中位数15个月）。
CSS患者出现支气管受累时可能存在哮喘。高分辨率CT上的磨玻璃影和实变影反映了嗜酸性肺炎、血管炎和肺泡出血的存在。TBLB对CSS的诊断有显著局限性。早期诊断和治疗可带来满意的预后。
