Fuger M, Clair M-P, El Ayoun Ibrahim N, L'Excellent S, Nizery L, O'Neill C, Tabone L, Truffinet O, Yakovleff C, de Blic J
Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
Arch Pediatr. 2016 May;23(5):525-31. doi: 10.1016/j.arcped.2016.02.020. Epub 2016 Mar 25.
Chronic interstitial lung disease (ILD) in children is a heterogeneous group of rare lung disorders characterized by an inflammatory process of the alveolar wall and the pulmonary interstitium that induces gas exchange disorders. The diagnostic approach to an ILD involves three essential steps: recognizing the ILD, appreciating the impact, and identifying the cause. The spectrum of clinical findings depends to a large extent on age. In the newborn, the beginning is often abrupt (neonatal respiratory distress), whereas there is a more gradual onset in infants (failure to thrive, tachypnea, indrawing of the respiratory muscles). In older children, the onset is insidious and the diagnosis can only be made at an advanced stage of the disease. The diagnosis is based on noninvasive methods (clinical history, respiratory function tests, chest X-ray, and high-resolution CT scan) and invasive techniques (bronchoalveolar lavage, transbronchial biopsy, video-assisted thoracoscopic biopsy, and open lung biopsy). The treatment of interstitial lung disease in children depends on the nature of the underlying pathology. The most common therapeutic approach involves the use of corticosteroids and immunosuppressive agents for their anti-inflammatory and antifibrotic effects. Children with ILD also need support therapy (oxygen therapy, nutritional support, treatment of pulmonary arterial hypertension, vaccination). Lung transplantation is discussed in patients with severe respiratory failure.
儿童慢性间质性肺疾病（ILD）是一组异质性罕见的肺部疾病，其特征为肺泡壁和肺间质的炎症过程，可导致气体交换障碍。ILD的诊断方法包括三个基本步骤：识别ILD、评估其影响以及确定病因。临床症状的范围在很大程度上取决于年龄。在新生儿中，起病通常较为突然（新生儿呼吸窘迫），而在婴儿中起病则较为缓慢（生长发育迟缓、呼吸急促、呼吸肌内陷）。在大龄儿童中，起病隐匿，往往在疾病晚期才能确诊。诊断基于非侵入性方法（临床病史、呼吸功能测试、胸部X线和高分辨率CT扫描）以及侵入性技术（支气管肺泡灌洗、经支气管活检、电视辅助胸腔镜活检和开胸肺活检）。儿童间质性肺疾病的治疗取决于潜在病理的性质。最常见的治疗方法是使用皮质类固醇和免疫抑制剂，利用其抗炎和抗纤维化作用。患有ILD的儿童还需要支持治疗（氧疗、营养支持、肺动脉高压治疗、疫苗接种）。对于严重呼吸衰竭的患者，会讨论肺移植问题。
