Hewitt C J, Hull D, Keeling J W
Arch Dis Child. 1977 Jan;52(1):22-37. doi: 10.1136/adc.52.1.22.
Fibrosing alveolitis is a rare, diffuse lung disease characterized by varying combinations of two histological features: thickening of alveolar walls and the presence of large mononuclear cells in the alveolar spaces. Clinical details of 10 children with fibrosing alveolitis are reported. The main symptoms in children are tachypnoea or dyspnoea, cough, poor weight gain, and cyanosis. The condition is similar to that in adults, but it is usually a more acute illness, and if untreated, more predictably fatal. Respiratory failure, pulmonary hypertension, and cardiac failure are the major complications. Less commonly, superimposed bacterial infection and pneumothorax occur. Chest x-rays often show a sequence of changes with a ground-glass appearance and fine mottling in the early stage of the disease, progressing to a picture of mainly hilar linear markings in those children who recover. The histological features at lung biopsy or necropsy are described; these correlated poorly with the radiological features, steroid responsiveness, and clinical course. Lung function tests in 3 older children showed evidence of markedly reduced lung volumes in 2. Static lung compliance in 4 children in the acute stage of the illness was normal in 3 and diminished in one. The response to steroid therapy was analysed in cases from the literature and the 10 reported cases. No spontaneous remissions occurred, all the survivors having been treated with corticosteroids. In children fibrosing alveolitis is almost always a corticosteroid-responsive disease. An appropriate course of prednisolone would be of at least 4 week's, but preferably of 8 weeks' duration, at a minimum daily dose of 2 mg/kg. After improvement the steroid withdrawal should be cautious and protracted, comprising at least a year's continuous treatment.
纤维化肺泡炎是一种罕见的弥漫性肺部疾病，其特征为两种组织学特征的不同组合：肺泡壁增厚和肺泡腔内存在大量单核细胞。本文报告了10例儿童纤维化肺泡炎的临床详情。儿童的主要症状为呼吸急促或呼吸困难、咳嗽、体重增加缓慢及发绀。该病情况与成人相似，但通常病情更为急性，若不治疗，更易致命。呼吸衰竭、肺动脉高压和心力衰竭是主要并发症。较少见的是并发细菌感染和气胸。胸部X光片常显示一系列变化，疾病早期呈磨玻璃样外观和细小结节，在恢复的儿童中进展为主要为肺门线状阴影的影像。描述了肺活检或尸检时的组织学特征；这些特征与放射学特征、类固醇反应性及临床病程相关性较差。3名年龄较大儿童的肺功能测试显示，其中2例肺容积明显减小。4例处于疾病急性期儿童的静态肺顺应性，3例正常，1例降低。分析了文献中的病例及本文报告的10例病例对类固醇治疗的反应。无一例自发缓解，所有幸存者均接受了皮质类固醇治疗。在儿童中，纤维化肺泡炎几乎总是一种对皮质类固醇有反应的疾病。泼尼松龙的适当疗程至少应为4周，但最好为8周，最小日剂量为2mg/kg。病情改善后，类固醇撤药应谨慎且缓慢，至少持续治疗一年。
