6例以肺部表现为首发症状原发性小血管炎的临床特点分析

Analysis of the clinical manifestations in six cases of primary polyangitis with initial symptoms related to lung

目的:探讨以肺部症状为首发表现的原发性小血管炎的临床特征和影像学表现。方法:对6例以肺部症状为首发表现并诊断为原发性小血管炎的患者进行回顾性分析,分析其临床表现、肺部影像学特征、实验室资料。结果:肺部受累的首发症状主要是咳嗽、咳痰、发热、气促、咯血、胸闷和胸痛。影像学特征性表现为肺部多发性斑片状影、间质纤维化或两肺弥漫性网格状改变。肺外其他系统伴随症状常见有血尿4例(其中1例为肉眼血尿),管型尿2例,贫血4例,高血压3例,皮肤损害1例,关节肌肉症状1例,肝功能异常3例,肾功能异常4例。全部病例抗中性粒细胞胞浆抗体阳性,且均为核周型。3例经肾活检确诊。结论:原发性小血管炎常累及肺部,且可以肺部为首发症状,其临床症状及肺部影像学表现多无特异性,亦常累及口、鼻、肾脏、皮肤等器官。

Objective To explore the clinical and radiological features of primary polyangitis, especially microscopic polyangitis with initial symptoms related to lung. Methods Six cases of primary polyangitis with initial symptoms related to lung were analyzed retrospectively. Results The initial respiratory symptoms included cough, excessive sputum, fever, dyspnea, haemoptysis, oppressed feeling in chest and chest pain. Respiratory failure did not occur. Chest imaging showed that multiple laminar opaque and interstitial fibrosis were common in those cases. Other symptoms included hematuria, albuminuria, anemia, hypertension, skin lesion, joint lesion, dysfunction of liver and kidney,etc. Antineutrophil cytoplasmic antibody was positive in all cases. Three underwent renal biopsy. Conclusion The lung is frequently involved in primary polyangitis. The clinical manifestations are unspecific. Careful attention on the mouth, ears, eyes, skin, chest imaging, antineutrophil cytoplasmic antibodies, renal function and even biopsy is important for making diagnosis.