Castleman病所致的与副肿瘤性天疱疮相关的闭塞性细支气管炎的临床、病理与影像

Bronchiolitis obliterans and paraneoplastic pemphigus associated with Castleman disease: radiologic features with clinical and histopathologic correlation

目的 探讨与Castleman病所致的副肿瘤性天疱疮 (PNP)相关的闭塞性细支气管炎 (BO)的临床、病理与影像学特征。方法 5例患者包括男 3例 ,女 2例 ,平均年龄 2 1岁。 4例BO患者经临床诊断 ,1例经病理证实。所有患者均行吸气相和呼气相X线胸片和高分辨率CT(HRCT)扫描检查。结果 5例患者分别在PNP出现 1～ 8个月后继发BO症状。胸片 4例表现为双肺透亮度增加和血管纹理稀疏等过度充气改变。HRCT显示为弥漫性空气潴留征者 5例、双肺多个亚段及其远端较大的细支气管柱状扩张 4例、小叶核心和支气管管壁增厚 4例、纵隔积气 3例、片状肺泡实变 (磨玻璃影 )2例。术后随访 5个月至 3年均预后不良 ,呈现不同程度的Ⅱ型呼吸衰竭症状。结论 BO是伴有PNP的Castleman病的少见而严重的合并症 ,是导致预后不良的主要原因。HRCT上 ,BO的HRCT最常见表现包括呼气相空气潴留征。

Objective To describe the clinical, histopathologic and radiological features of bronchiolitis obliterans associated with paraneoplastic pemphigus and Castleman disease. Methods The patients were 3 men and 2 women with a mean age of 21 years. The clinical and pathologic records and radiological findings in 5 clinically proven cases of BO with PNP were reviewed. Results Five patients developed BO 1 to 8 months after the onset of PNP. The chest radiograph showed slight or severe hyperinflation of bilateral lung in 4 patients. HRCT findings included diffuse air trapping in 100%, subsegmental bronchial dilatation in 80%, bronchial wall thickening in 80%, pneumomediastinum in 60%, and patchy alveolar consolidation or ground-glass opacity in 40%. Followed-up from 5 months to 3 years after surgery showed that all patients developed type Ⅱ respiratory failure with poor prognosis. Conclusion As a rare and severe complication, BO is a major factor that limits the prognosis of PNP associated with Castleman disease. Air trapping, bronchial dilatation, and bronchial wall thickening were most common findings on HRCT in patients with BO.