Table 1: Childhood interstitial lung disease classification.

Specific interstitial lung diseases in children

Non-specific interstitial lung diseases in children

A. Diffuse developmental disorders

A. Normal host disease

1. Acinar dyskinesia

2. Congenital alveolar dysplasia

3. Alveolocapillary dysplasia with poor alignment of the pulmonary veins

1. Infectious and post-infectious processes

2. Environmental agent-related disorders:

• Hypersensitivity pneumonia

• Toxic inhalation

3. Aspiration syndrome

4. Eosinophilic pneumonia

B. Growth abnormalities

B. Systematic diseases with pulmonary involvement

1. Pulmonary hypoplasia

2. Chronic neonatal lung disease:

• Bronchopulmonary Dysplasia

• Acquired chronic lung disease in term newborn

3. Lung structural changes related to chromosomal abnormalities:

• Trisomy 2

• Others

4. Associated with congenital heart disease in chromosomally normal children

1. Immune-related diseases

2. Deposit Diseases

3. Sarcoidosis

4. Langerhans cell histiocytosis

5. Neoplastic infiltrates

C. Specific conditions of undefined etiology

C. Diseases of the immunocompromised host

1. Pulmonary interstitial glycogenosis

2. Childhood neuroendocrine cell hyperplasia

1. Opportunistic infections

2. Related to therapeutic intervention

3. Related to transplantation and rejection

4. Diffuse alveolar damage of unknown etiology

D. Disease due to defects in surfactant function

D. Diseases that mimic interstitial diseases

1. Surfactant Protein B mutations: dominant histological pattern, pulmonary alveolar proteinosis, and variants

2. Surfactant protein C mutations: dominant histological pattern, chronic pneumonitis of childhood; also desquamative and nonspecific interstitial pneumonia

3. ABCA3 mutations: dominant histological pattern, pulmonary alveolar proteinosis and variants. Also chronic pneumonitis of childhood, desquamative interstitial pneumonia, nonspecific interstitial pneumonia

4. Histology compatible with surfactant defects, but without recognized genetic etiology:

• Alveolar pulmonary proteinosis

• Chronic pneumonitis of childhood

• Desquamative interstitial pneumonitis

• Nonspecific interstitial pneumonia

1. Arterial hypertensive vascular disease

2. Congestive vascular disease including veno-occlusive disease

3. Congestive changes related to cardiac dysfunction

4. Lymphatic disorders

Adapted from: Kurland G, Deterding RR, et al. American Thoracic Society Committee on Childhood Interstitial Lung Disease (chILD) and the chILD Research Network. An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy.