Updated: 12/15/2019

Interstitial Lung Disease

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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
  • Prognosis
    • variable and depends on the underlying cause
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
 

 

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(M2.PL.17.4867) A 57-year-old man presents to his primary care physician with shortness of breath. The patient has had worsening shortness of breath for the past year. He has a past medical history of asthma and constipation. His current medications include albuterol and sodium docusate. The patient was an officer in the navy and currently lives at home with his wife. He has a 52 pack-year smoking history. Pulmonary function testing is performed at this visit as revealed in Figure A. The results of this test at this visit are revealed by the blue loop, as compared to his results 12 years ago as seen in the green dotted loop of Figure A. Which of the following is a possible diagnosis for this patient's presentation? Tested Concept

QID: 109613
FIGURES:
1

Chronic obstructive pulmonary disease

11%

(7/63)

2

Unstable angina

0%

(0/63)

3

Interstitial lung disease

87%

(55/63)

4

Poorly treated asthma

0%

(0/63)

5

Small cell carcinoma of the lung

0%

(0/63)

M 6 D

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