普通型间质性肺炎与非特异性间质性肺炎急性加重的临床与病理表现

目的 研究间质性肺疾病患者急性加重的临床和病理表现.方法 回顾性总结北京协和医院自1999年4月至2007年6月间经肺部病理活检证实的普通型间质性肺炎(UIP)和非特异性间质性肺炎(NSIP)急性加重患者各3例的临床资料.其中5例为急性加重前的间质性肺炎病理的结果,1例为急性加重后的尸检结果.结果 6例中男2例,女4例,年龄29～57岁(中位年龄51岁).急性加重前的病理表现:3例为UIP(1例为特发性,1例为皮肌炎继发,1例为结缔组织病继发),2例为NSIP(1例为特发性,1例为皮肌炎继发),1例尸检为弥漫性肺泡损伤合并NSIP(皮肌炎继发).6例中2例在急性加重前1周曾行胸腔镜肺活检术,5例发热,2例白细胞数增高,5例中性粒细胞比例升高.6例的痰培养和血培养无阳性发现.急性加重时氧合指数为200 mm Hg(四分位间距为158～237 mm Hg,1 mm Hg=0.133 kPa),4例ESR增快,2例C反应蛋白升高.胸部CT示在原有病变基础上出现磨玻璃影和实变影.5例使用糖皮质激素35～1000 mg/d,2例使用静脉人血丙种球蛋白,住院期间死亡4例,3例使用有创机械通气的患者均死亡,存活出院者1例稳定,1例好转,其后2例均失访.结论 UIP和NSIP都可出现急性加重,临床表现为突然出现的呼吸困难及发热,无特异性,诱因不清,广谱抗生素治疗无反应,大剂量糖皮质激素及静脉人血丙种球蛋白治疗可能有效.

Acute exacerbation of usual interstitial pneumonia and nonspecific interstitial pneumonia:analysis of 6 cases

OBJECTIVE: Acute exacerbation of diffuse parenchymal lung disease (DPLD) is a condition in which patients with usual interstitial pneumonia (UIP), and other forms of interstitial lung disease, develops rapid respiratory failure, accompanied by extensive radiological infiltrates, and had no evidence of infection. The pathologic features of this condition are usually diffuse alveolar damage (DAD) and the outcome is poor. Our study was to define the clinicopathologic features and outcome of acute exacerbation in 3 patients with UIP and 3 with nonspecific interstitial pneumonia (NSIP). METHOD: The clinical data of the 6 patients from April 1999 to Jun 2007 were analyzed retrospectively. RESULTS: In the 6 patients, 2 were males. The median age was 51 yrs (29 -57 yrs). Three case had UIP 1 UIP/idiopathic pulmonary fibrosis (IPF), 1 UIP/dermatomyositis (DM), 1 UIP/UCTD], 2 had NSIP (1 idiopathic NSIP, 1 NSIP/DM), and 1 was diagnosed as DAD (the basic pathology was NSIP/DM) by autopsy. Four of the patients underwent video-assisted thoracoscopic surgery (VATS) for diagnosis, and 1 underwent CT-guided transthoracic needle biopsy. Two of them underwent surgical lung biopsy 1 week before acute exacerbation. Five cases had fever. Computed tomography data were available in all cases and showed the presence of extensive bilateral ground-glass opacities (5/6), sometimes accompanied by focal consolidation (2/6), superimposed on underlying fibrosis. The median oxygenation index was 200 (quartile range: 158-237) mm Hg (1 mm Hg = 0.133 kPa). Five patients were treated with corticosteroids, and in some combined with cyclophosphamide or intravenous immunoglobulin. Two patients survived the acute episode and were discharged from hospital. All 3 patients using mechanical ventilation died. CONCLUSIONS: Both UIP and NSIP can develop acute exacerbations. The trigger of acute exacerbation was unclear, but in some cases it maybe related to VATS. The clinical features include rapid respiratory failure, accompanied by extensive radiological infiltrates. The fatality is high. Corticosteroids or intravenous immunoglobulin may be helpful in the treatment of the condition.