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Children’s Interstitial Lung Disease
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(diffuse infiltrative pathologies, PID acronym in French of Pneumopathies Interstitielles Diffuses, ChILD syndrome)
Heterogeneous group of rare respiratory diseases characterized by decreased lung function, reduce blood oxygen levels and diffuse pulmonary infiltrates on imaging. A genetic cause is found in about 20 % of cases.
Symptomatology:
- newborn: early severe respiratory distress in a full-term baby, diffuse infiltrates to chest Xray, no cardiac disease
- infant: progressive onset, dyspnea on exertion (bottle feeding), dry cough, wheezing, polypnea, indrawing
- child: slow onset, dyspnea at exercise and rest, clubbing, chronic respiratory failure
Causes: (see corresponding items)
Idiopathic diffuse interstitial lung disease
- idiopathic pulmonary fibrosis
- desquamative interstitial lung disease
- nonspecific interstitial lung disease
- lymphocytic interstitial lung disease
- cryptogenic organized pneumopathy
- interstitial lung disease with immune deficiency
- respiratory bronchiolitis with diffuse interstitial lung disease
- idiopathic pleuropulmonary fibrosis
- combined pulmonary emphysema-pulmonary fibrosis
- familial pulmonary fibrosis
Diffuse interstitial lung disease caused by abnormalities of the pulmonary surfactant metabolism
♦ diffuse interstitial lung disease by genetic abnormality of the pulmonary surfactant
- diffuse interstitial lung disease by ABCA3 deficiency
- diffuse interstitial lung disease by SP-A deficiency
- diffuse interstitial lung disease by SP-A1 deficiency
- diffuse interstitial lung disease by SP-A2 deficiency
- diffuse interstitial lung disease by SP-B deficiency
- diffuse interstitial lung disease se by SP-C deficiency
- brain-lung-thyroid syndrome (NKX2-1)(TTF1)
♦ pulmonary alveolar proteinoses
- autoimmune pulmonary alveolar proteinosis
- pulmonary alveolar proteinosis by pulmonary CSF2RA deficiency
- pulmonary alveolar proteinosis by CSF2RB deficiency
- severe pulmonary alveolar proteinosis by MARS deficiency
- other pulmonary alveolar proteinosis
Hyperplasia of the neuroendocrine cells in infancy
Alveolar pulmonary microlithiasis
Eosinophilic pneumonitis
- idiopathic eosinophilic pneumonitis
- eosinophilic pneumonitis of parasitic origin
- eosinophilic pneumonitis of non-parasitic infectious origin
- eosinophilic pneumonitis due to medications
- idiopathic hypereosinophilic bronchiolitis
- idiopathic hypereosinophilic syndrome
- primary hypereosinophilic syndrome (myeloid or lymphocytic)
- other eosinophilic pneumonitis
Other Interstitial lung disease
- respiratory bronchiolitis with interstitial lung disease
Chronic lung diseases associated with granulomatous systemic diseases
- chronic granulomatosis
- Langerhans granulomatosis
- eosinophilic granuloma of the bones
- histiocytosis X
- Hand-Schüller-Christian disease
- Letterer-Siwe disease
- Hashimoto-Pritzker syndrome
- sarcoidosis
- Blau syndrome
Chronic lung diseases associated with systemic diseases with metabolic involvement
- familial hypocalciuric hypercalcemia
- familial hypocalciuric hypercalcemia type 1
- familial hypocalciuric hypercalcemia type 2
- familial hypocalciuric hypercalcemia type 3
- Gaucher disease type 1
- Gaucher disease type 2
- Gaucher disease type 3
- Niemann-Pick disease type B
- Niemann-Pick disease type C
- Hermansky-Pudlak syndrome with pulmonary fibrosis
- pulmonary glycogenosis
Chronic lung diseases associated with systemic autoimmune and inflammatory diseases
- mixed connectivitis
- interstitial lung disease secondary to a crude or undifferentiated connectivitis (IPAF)
- dermatomyositis
- systemic lupus erythematosus
- rheumatoid arthritis
- polymyositis
- inflammatory idiopathy myopathy
- systemic sclerodermia
- hereditary sclerosing poikiloderma with pulmonary and tendinous involvement (FAM111B)
- autoimmune syndrome of interstitial pneumonitis-arthritis (COPA deficiency)
- Gougerot Sjögren's syndrome
- atrophicans polychondritis
Chronic lung diseases associated with systemic diseases with vasculitis
- glomerular basal membrane antibody disease (Goodpasture syndrome)
- vasculitis associated with cytoplasmic antineutrophilic antibodies
- granulomatosis with polyangiitis (Wegener syndrome)
- Churg Strauss syndrome
- microscopic polyangiitis
- cryoglobulinemic vasculitis
- mixed cryoglobulinemia type II
- mixed cryoglobulinemia type III
- vasculitis with immunoglobulin A
- vasculopathy associated with STING ( TMEM 173) or SAVI
Intraalveolar hemorrhages
- autoimmune intraalveolar hemorrhage
- Heiner syndrome
- Lane Hamilton syndrome
- non-immune intraalveolar hemorrhage
- idiopathic pulmonary hemosiderosis
Amyloidosis
Diagnostic criteria:
3 clinical and/or radiological criteria out of the following 4 must be present:
- respiratory symptoms
- clinical signs of respiratory failure
- decreased oxygen saturation (SpO2) and/or hypoxemia
- diffuse parenchymal involvement on the chest X-ray or CT scan
Treatments:
Oxygen therapy:
and corticosteroid therapy (usually not very effective), hydroxychloroquine, azithromycin. Nutritional needs are increased (120 %); gavage by nasogastric tube or gastrostomy is almost always the rule
Anesthetic implications:
SpO2, at room air; chest XRay, echocardiography (heart disease, pulmonary hypertension, impact on RV function), ongoing treatment, gentle ventilation (permissive hypercapnia)
References:
- www.europeanlunginfo.org/child
- Epaud R, Delestrain C, Nattes E, Nathan N.
Démarche diagnostique devant une pneumopathie interstitielle.
Arch Fr Pédiatr 2017 ; S : 26-8
- HAS (France). Centre de Référence des Maladies Respiratoires Rares RespiRare. Protocole National de Diagnostic et de Soins (PNDS) : Pneumopathies interstitielles diffuses de l’enfant. Octobre 2017
Updated: October 2021