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Restrictive Pulmonary Disease

Idiopathic pulmonary fibrosis

• The development of scar tissue within the lungs

Clinical presentation

• Dyspnea
• Dry cough
• Chest discomfort

Labs, Studies and Physical Exam Findings

• Fine crackles at the bases may be present
• FEV1 is reduced
• FVC is reduced
• Lung volumes are reduced
• Chest x-ray may not be helpful
• CT
• Wedge biopsy

Treatment

• Prednisone may help
• Oxygen improves symptoms
• Lung transplant

Pneumoconiosis

• A group of diseases which are restrictive and caused by inhalation of specific types of dust.
  • Black lung or coal miners lung = coal
  • Asbestosis = asbestos
  • Silicosis = silica
  • Berylliosis = beryllium associated with aerospace manufacturing

Clinical Presentation

• Shortness of breath
• Cough

Labs, Studies and Physical Exam Findings

• Inspiratory rales
• FEV1 is reduced
• FVC is reduced
• Lung volumes are reduced
• Chest x-ray may be helpful
• Silicosis – 2-5 mm nodules in middle and upper lung fields. Eggshell calcifications
• Coal miners lung – nodular opacities in upper lung fields. Eggshell calcifications
• Asbestosis – irregular parenchymal opacities and pleural abnormalities. These are typically at the bases of the lungs.
• CT

Treatment

• Oxygen will alleviate symptoms
• Do not smoke!
• Avoid dust of any type

Sarcoidosis

• An inflammatory disease which is characterized by granulomas throughout the body. The lungs are by far the most common site.

Clinical Presentation

• Fever
• Dyspnea
• Routine physical with findings on chest x-ray
• Fatigue
• Weight loss
• Dry cough
• Skin rashes

Labs, Studies and Physical Exam Findings

• Leukopenia
• Decreased lung volume
• Chest x-ray
  • Hilar adenopathy
  • Nodules or infiltrates in the parenchyma as in later disease
• Biopsy showing noncaseating granulomas is diagnostic
• EKG

Treatment

• In most cases observation is the first step
• Corticosteroids

<< Click here to get 40 Pulmonology questions straight from my book The Final Step >>