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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Chronic interstitial lung disease in children: Diagnostic approach and management]
M Fuger1, M-P Clair1, N El Ayoun Ibrahim1
1Service de pneumologie pédiatrique, hôpital Necker-Enfants-Malades, AP-HP, université Paris Descartes, 149, rue de Sèvres, 75015 Paris, France.
Insights
Interstitial lung disease (ILD) in children presents diverse symptoms based on age, requiring a multi-step diagnostic approach. Treatment focuses on anti-inflammatory medications and supportive care, with lung transplantation as a last resort for severe cases.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Disease
Background:
- Chronic interstitial lung disease (ILD) in children is a rare, heterogeneous group of disorders.
- It involves inflammation of the alveolar wall and interstitium, leading to impaired gas exchange.
- Clinical presentation varies significantly with age, from neonatal respiratory distress to insidious onset in older children.
Purpose of the Study:
- To outline the diagnostic and therapeutic strategies for pediatric interstitial lung disease.
- To emphasize the age-dependent clinical manifestations and diagnostic challenges.
- To review current treatment modalities and supportive care for children with ILD.
Main Methods:
- Diagnosis relies on a combination of noninvasive methods including clinical history, pulmonary function tests, chest X-ray, and high-resolution CT scans.
- Invasive techniques such as bronchoalveolar lavage and various biopsy methods are employed for etiological identification.
- Assessment considers the impact of the disease and aims to identify the underlying cause.
Main Results:
- Diagnostic approach involves recognition, impact assessment, and etiological identification.
- Age influences symptom onset and presentation, impacting diagnostic timelines.
- Treatment strategies are tailored to the specific pathology, often involving corticosteroids and immunosuppressants.
Conclusions:
- Pediatric ILD diagnosis requires a systematic approach integrating noninvasive and invasive methods.
- Management necessitates individualized treatment plans, including anti-inflammatory agents, supportive care, and consideration of lung transplantation for severe cases.
- Early recognition and appropriate intervention are crucial for managing this rare group of pediatric lung disorders.
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.
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