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Updated: Dec 24 2021

Interstitial Lung Disease

  • Snapshot
    • A 68-year-old man presents to the emergency department for worsening shortness of breath. His dyspnea was most notable with exertion; however, over the course of the past several months, it presented at rest. He reports that he has a non-productive cough and denies have any sinus pain, fever, malaise, chills, or night sweats. He has a past medical history of hypertension and hyperlipidemia. He is a retired aircraft mechanic and has smoked 1 pack of cigarettes daily for the past 45 years. His temperature is 98.6°F (37°C), blood pressure is 134/90 mmHg, pulse is 106/min, respirations are 23/min, and oxygen saturation is 88% on room air. On physical exam, the patient is alert to person but not place or time. There are rales on pulmonary auscultation and digital clubbing. A chest radiograph demonstrates a reticular pattern and honeycombing.
  • Introduction
    • Definition
      • inflammatory lung process that leads to alveolar wall thickening, impairing gas exchange
  • Etiology
    • Environmental
      • coal worker pneumoconiosis
      • silicosis
      • asbestosis
        • associated with roofing work, navy shipyard work 
        • associated with pleural plaques on chest imaging
        • may see ferruginous bodies on histology
          • asbestos fibers coated with iron and calcium
      • berylliosis
    • Granulomatous disease
      • sarcoidosis
      • granulomatosis with polyangiitis
      • eosinophillic granulomatosis with polyangiitis
      • histiocytosis x
    • Alveolar filling disease
      • Goodpasture syndrome
      • alveolar proteinosis
      • pulmonary hemosiderosis
    • Hypersensitivity lung disease
      • eosinophilic pneumonitis
      • hypersensitivity pneumonitis
    • Drugs
      • amiodarone
      • bleomycin
      • phenytoin
    • Pathogenesis
      • distal airspace tissue injury (due to a myriad of causes) leads to abnormal wound healing
        • causes structural remodeling, where collagenous fibrosis ensues, impairing gas exchange
  • Presentation
    • Symptoms and physical exam findings differ depending on the underlying cause; for example, patients with pulmonary sarcoidosis may have erythema nodosum
    • Symptoms
      • dyspnea (initially with exertion and as the disease progresses, then at rest)
      • cough
      • fatigue
    • Physical exam
      • rales on pulmonary auscultation
      • digital clubbing
      • cyanosis in advanced disease
  • Imaging
    • Chest radiography
      • indication
        • typically initial imaging option in the workup of interstitial lung disease
      • findings
        • reticular pattern
        • honeycombing
    • High-resolution CT scan of the chest
      • indication
        • diagnostic and helps narrow the cause of interstitial lung disease
  • Studies
    • Pulmonary function tests
      • indication
        • obtained in virtually all patients with interstitial lung disease
          • aids in assessing severity of lung disease and determines whether there is an obstructive, restrictive, or mixed lung patter
  • Differential
    • Heart failure
      • differentiating factors
        • patients may have peripheral edema and hemoptysis
    • Pulmonary embolism
      • differentiating factors
        • patients will have a filling defect on CT angiogram of the chest
  • Treatment
    • Of note, treatment is dependent upon the underlying cause
    • Conservative
      • smoking cessation and influenza and pneumococcal vaccines
        • indication
          • should be given to all patients with interstitial lung disease, unless contraindicated
    • Medical
      • intravenous corticosteroids
        • indication
          • first-line therapy for patients for acute respiratory therapy
      • intravenous cyclophosphamide
        • indication
          • second-line therapy for patients for acute respiratory therapy
  • Complications
    • Irreversible pulmonary fibrosis
    • Respiratory failure
  • Prognosis
    • Variable and depends on the underlying cause
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