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过敏性肉芽肿性血管炎的病理学及高分辨CT表现

发布时间:2012-05-15    点击数:

摘要 目的:研究过敏性肉芽肿性血管炎(CSS)的肺部受累,来明确期临床特点,肺部病理表现及对治疗的反应和预后。

方法:回顾分析了16例CSS患者的临床资料、临床表现、超薄CT及病理。所有患者均接受了经支气管肺活检(TBLB)。6例患者同时还接受了开胸肺活检。

结果:16例CSS患者包括男性7例,女性9例,年龄自14岁到61岁(中位年龄47.5岁)。胸腔外脏器受累包括神经系统 (7/16) 和皮肤(5/16)。呼吸道症状包括咳嗽 (12/16),劳累性呼吸困难 (11/16),咯血(4/16)和胸痛 (3/16)。 CT 表现包括双肺毛玻璃样混着(12/16), 双 patchy 混着 (12/16),和中央小叶结节 (6/16)。 TBLB 病理检查提示d嗜酸细胞增加 (3/16),血管炎 (3/16)和间质性肺炎 (16/16)。开胸肺活检病理表现为4例有坏死性血管炎, 5例有微血管炎, 3有嗜酸细胞性肺炎, 2例可见肉芽肿, 3例有气道异常。所有患者在治疗后症状好转(3 至 51 个月; 平均 15个月).

结论:当CSS患者有支气管受累时,可以出现哮喘。高分辨CT出现毛玻璃样混着和实变提示存在嗜酸细胞性肺炎、血管炎和肺泡出血。TBLB 对CSS诊断有很大的局限性。早期诊断和治疗预后较好。

附原文: OBJECTIVE:To investigate the (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

引自:Feng RE, Xu WB, Shi JH, Mahmoudi A, Mu WB, Zheng WJ, Zhu YJ, Liu HR. Pathological and high resolution CT findings in Churg-Strauss syndrome. Chin Med Sci J. 2011 Mar;26(1):1

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