title: [Chronic interstitial lung disease in children: Diagnostic approach and management].
abstract: 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.
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France. Electronic address: fuger.marilyn@gmail.com.
authors.forename: M
authors.initials: M
authors.lastname: Fuger
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: M-P
authors.initials: MP
authors.lastname: Clair
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: N
authors.initials: N
authors.lastname: El Ayoun Ibrahim
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: L'Excellent
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Nizery
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: O'Neill
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Tabone
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: O
authors.initials: O
authors.lastname: Truffinet
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Yakovleff
authors.affiliation: Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: de Blic
mesh_terms: D002648:Child; D002908:Chronic Disease; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000145:classification / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D010100:Oxygen / Q000627:therapeutic use*; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D010100:Oxygen