PANCE Blueprint Pulmonary (10%)

Restrictive Pulmonary Disease (PEARLS)

The NCCPA™ PANCE  Pulmonary Content Blueprint covers three types of restrictive pulmonary diseases

Idiopathic pulmonary fibrosis Chronic progressive lung disorder characterized by increasing scarring, which ultimately reduces the capacity of the lungs; etiology unknown

  • Physical exam: Inspiratory crackles
  • CT: Diffuse, patchy fibrosis with pleural honeycombing, reticular opacities

FEV1/FVC ratio: Increased

Pneumoconiosis Any fibrosis of the lung tissues caused by dust in the lungs after prolonged environmental or occupational contact

  • Asbestosis: shipbuilding and chest x-ray findings of interstitial fibrosis and pleural thickening
  • Silicosis: mines and "eggshell" calcification of hilar lymph nodes
  • Berylliosis: aerospace, electrical plants
  • Coal Worker's Pneumoconiosis: coal miners, CXR demonstrates nodular opacities
 Sarcoidosis Chronic autoimmune inflammatory disease in which small nodules (granulomas) develop in lungs, lymph nodes, and other organs

↑ ACE levels + Bilateral hilar adenopathy

Presentation:

  • Pulmonary manifestations (most common), erythema nodosum, parotid gland enlargement
  • Chest radiograph: Bilateral hilar lymphadenopathy. Reticular infiltrates.
  • Biopsy: non-caseating granulomas

Treatment: Steroids

Lesson Intro Video

Pulmonary hypertension (Prev Lesson)
(Next Lesson) Idiopathic pulmonary fibrosis
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