Interstitial Lung Disease

Interstitial Lung Disease is a topic covered in the Washington Manual of Medical Therapeutics.

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General Principles

Definition

ILDs are a heterogeneous group of disorders, pathologically characterized by infiltration of the lung interstitium with cells, fluid, and/or connective tissue.

Classification

  • ILD of known etiology
    • Medication (e.g., bleomycin, amiodarone, nitrofurantoin, methotrexate)
    • CTD (e.g., rheumatoid arthritis, scleroderma, Sjögren syndrome, antisynthetase syndrome)
    • Pneumoconiosis (e.g., coal worker’s pneumoconiosis, silicosis, asbestosis)
    • Radiation
    • Lymphangitic carcinomatosis
  • Idiopathic interstitial pneumonias1
    • Major idiopathic interstitial pneumonias:
      • Idiopathic pulmonary fibrosis (IPF) (idiopathic usual interstitial pneumonia [UIP])
      • Idiopathic nonspecific interstitial pneumonia (NSIP)
      • Desquamative interstitial pneumonia (DIP)
      • Respiratory bronchiolitis interstitial lung disease (RB-ILD)
      • Cryptogenic organizing pneumonia (COP) (idiopathic organizing pneumonia)
      • Acute interstitial pneumonia
    • Rare idiopathic interstitial pneumonias:
      • Idiopathic lymphoid interstitial pneumonia (LIP)
      • Idiopathic pleuroparenchymal fibroelastosis (IPPFE)
    • Unclassifiable idiopathic interstitial pneumonia
  • Granulomatous ILD
    • Sarcoidosis
    • Hypersensitivity pneumonitis (HP)
  • Cystic lung disease
    • Lymphangioleiomyomatosis (LAM)
    • Pulmonary Langerhans cell histiocytosis (PLCH)
    • Genetic diseases (Birt–Hogg–Dubé, tuberous sclerosis)
    • Light chain deposition disease

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