Table 1.
Classification of pediatric interstitial lung disease by etiology.
| 1) Interstitial lung disease observed mainly in infancy |
| − Developmental disorders and growth abnormalities |
| + Acinar dysplasia |
| + Alveolar-capillary dysplasia with pulmonary vein misalignment |
| + Primary alveolar growth abnormalities: congenital alveolar dysplasia / pulmonary hypoplasia / bronchopulmonary dysplasia |
| + Structural alveolar anomalies due to congenital heart disease or chromosomal abnormalities |
| − Genetic surfactant dysfunctions |
| + SPFTB gene mutations |
| + SPFTC gene mutations |
| + ABCA3 gene mutations |
| + NKX2.1 gene mutations |
| − Conditions specific to infancy |
| + Pulmonary interstitial glycogenesis |
| + Neuroendocrine cell hyperplasia of infancy |
| 2) Interstitial lung disease related to a primary systemic disease |
| − Autoimmune diseases |
| + Rheumatoid arthritis |
| + Systemic sclerosis |
| + Systemic lupus erythematosus |
| + Sjögren syndrome |
| + Dermatomyositis and polymyositis |
| + Ankylosing spondylitis |
| + Mixed connective tissue disease |
| − Pulmonary vasculitis |
| + Wegener’s granulomatosis |
| + Churg-Strauss syndrome |
| + Microscopic polyangitis |
| + Goodpasture syndrome |
| + Henoch-Schönlein purpura |
| + Cryoglobulinemic vasculitis |
| − Granulomatous diseases |
| + Sarcoidosis |
| + Post-infectious chronic granulomatous diseases |
| + Histiocytosis X |
| + Pulmonary vasculitis (see above) |
| − Langerhans cell histiocytosis |
| − Metabolic disorders |
| + Lysosomal diseases (Gaucher’s disease, Niemann-Pick disease, Hermansky-Pudlak syndrome) |
| + Familial hypercalcemia with hypocalciuria |
| − Others |
| + Eosinophilic lung diseases |
| + Malignacy |
| + Inflammatory bowel diseases and celiac disease |
| + Primary biliary cirrhosis and chronic hepatitis |
| + Neurocutaneous disorders (tuberous sclerosis, neurofibromatosis) |
| + Amyloidosis |
| + Transplantation related interstitial lung disease and graft-versus-host disease |
| 3) Exposure-related Interstitial lung disease |
| − Hypersensitivity pneumonitis |
| − Interstitial lung disease caused by environmental factors (pollution, tobacco, toxic inhalation) |
| − Post-infectious non-granulomatous interstitial lung disease |
| − Aspiration |
| − Interstitial lung disease following medical treatments (radiation, drugs) |