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Updated: Jan 11 2022

Other Restrictive Etiologies

  • Collagen Vascular Disease
    • Systemic lupus erythematosus
      • pleuritis + pleural effusions
    • Rheumatoid arthritis (rheumatoid lung disease)
      • pleuritis + pleural effusions
      • also see pulmonary fibrosis (restrictive pattern)
      • bilateral, diffuse appearance on chest radiograph
        • progress to honeycomb lung in severe disease
      • presentation
        • gradual onset dyspnea
        • end-inspiratory rales at lung base
        • lung biopsy shows patchy interstitial lymphoid infiltrate into walls of alveolar units
      • NOT associated with rheumatoid nodules in the lung
        • this finding is associated with Caplan's syndrome (see Pneumoconioses )
    • Systemic sclerosis (scleroderma)
      • may also present with CREST syndrome (a variant of scleroderma)
      • interstitial fibrosis due to deposition of collagen
        • triggered by increased TGF-beta secreting T-cells accumulating in lungs
        • mainly involve capillaries and small arterioles
      • may lead to pulmonary hypertension and cor pulmonale
  • Iatrogenic Causes
    • Drug-associated
      • anti-cancer agents
        • bleomycin/busulfan, methotrexate, nitrosourea, and cyclophosphamide
      • anti-arrhythmics
        • amiodarone
    • Radiation-induced lung injury
      • post-treatment pneumonitis (1-6 months following)
        • associated with fever, dyspnea, and pleural effusions
      • radiation fibrosis (6-12 months following)
        • dyspnea, nonproductive cough, fine crackles, and pulmonary fibrosis on chest radiograph
  • Occupational Causes
    • Silicosis
      • associated with occupational exposures of sandblasting, mining, and stone fabrication
    • Silo filler's disease
      • hypersensitivity pneumonitis to nitrogen oxide gases released by plant matter
    • Byssinosis
      • hypersensitivity penumonitis to textile dusts (including cotton, hemp, and linen)
    • Farmer's lung
      • hypersensitivity pneumonitis to Saccharopholyspora rectivirgula (thermophilic actinomyes)
        • found in moldy hay
        • type III hypersensitivity reaction with antigen-antibody complex depositing in lung
        • can become type IV hypersensitivity reaction with chronic exposure
  • Idiopathic Pulmonary Fibrosis
    • Introduction
      • most common group of idiopathic interstitial pneumonia
      • chronic alveolitis with no known cause
      • persistent inflammation results in fibrosis (due to fibroblast proliferation) and cyst formation that is most prominent in subpleural regions (lower lung predominantly)
      • typically seen in men 40-70 years old
      • requires transplant at most advanced stages
    • Presentation
      • dyspnea on exertion most common symptom
      • gradual onset of dry cough
        • subpleural cystic enlargement = "honeycomb" lung
      • diagnosis can be made by HRCT if underlying causes excluded
        • surgical lung biopsy shows usual interstitial pneumonia (UIP)
    • Treatment
      • Nintedanib
        • receptor blocker of tyrosine kinases that mediate action of platelet-derived growth factor (PDGF), vascular endothelial growth factor (VEGF), fibroblast growth factor (FGF)
      • Pirfenidone
        • inhibitor of transforming growth factor-beta (TGF-beta)
  • Pulmonary Alveolar Proteinosis
    • Idiopathic pathology where proteinaceous material fills the avleoli
      • can be caused by primary (idiopathic) or secondary (infectious/malignancies) causes
    • Pathophysiology
      • defect in clearance (macrophages) or production of surfactant
        • macrophage defect and atypical infections sometimes seenin this pathology
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