特发性肺上叶纤维化一例报告及文献复习

Idiopathic Pulmonary Upper Lobar Fibrosis:One Case Report and Literature Review

目的提高对特发性肺上叶纤维化(IPUF)的认识。方法对1例IPUF患者的临床资料进行分析,并结合相关文献进行复习。结果患者男性,76岁,主因"间断咳嗽、气促1年,加重1个月"而入我院。外院曾予醋酸泼尼松龙30 mg/d治疗3个月无效。肺功能显示限制性通气功能障碍和肺弥散功能下降,并在1年内明显恶化。胸部HRCT显示双肺上叶胸膜及胸膜下小叶间隔明显增厚,中下肺叶病变较轻。相关的免疫指标如抗核抗体谱、抗中性粒细胞胞浆、类风湿因子及人类白细胞相关抗原B27等均为阴性。支气管刷片及肺泡灌洗液抗酸染色均为阴性。临床除外了溃疡性结肠炎、强直性脊柱炎、过敏性肺炎等疾病,因此临床诊断为IPUF。患者拒绝开胸肺活检,1年后死于呼吸衰竭。结论 IPUF是一个少见而独特的特发性肺纤维化类型,常伴有肺通气功能的急剧恶化,影像学表现为双肺上叶为主的肺纤维化和胸膜增厚。IPUF进展较快,预后较差,除肺移植外尚无有效治疗。

Objective To highlight the characteristics of idiopathic pulmonary upper lobar fibrosis （ IPUF） . Methods One patient with IPUF was presented and the related literatures were reviewed. Results A 76-year-old man was referred to our hospital because of intermittent cough and progressive shortness of breath for 1 year. The patient had been treated by prednisolone 30mg/d for 3 months and had no response. Chest HRCT showed bilateral thickened pleura and sub-pleural interlobular septa with moderate fibrotic changes mainly in the upper lobes. Pulmonary function displayed restrictive ventilatory dysfunction and decreased pulmonary diffusion capacity, which significantly deteriorated in 1 year （ FVC declined by 17% and diffusing capacity decreased by 20% ） . Serum antinuclear antibodies, anti-neutrophil cytoplasmic antibodies, rheumatoid factor and human leukocyte antigen B27 were all negative. Acid-fast staining of bronchial brushings and lavage fluid was negative. So the patient was clinically diagnosed of IPUF. He refused open lung biopsy, then died 1 year later. Conclusions IPUF is a rare and unique type of idiopathic interstitial lung diseases accompanied by a rapid deterioration of pulmonary function. Chest HRCT showed thickened pleura and sub-pleural interlobular septa mainly in bilateral upper lobes. IPUF progresses rapidly and prognosis is poor. So far there is no effective treatment except for lung transplantation.