PANCE Blueprint Pulmonary (10%)

Restrictive Pulmonary Disease (PEARLS)

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

osmosis Osmosis
Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason

  • Over time, the scarring gets worse and it becomes hard to take in a deep breath and the lungs cannot take in enough oxygen
  • Etiology unknown; MC of all interstitial lung diseases
  • In order to be considered "idiopathic", you must rule out other common causes such as drugs (amiodarone), and environmental or occupational exposures
    • non-idiopathic: smoking, viral infections, environmental (silica, hard metal dust), medication, genetics, XRT, GERD
  • Physical exam: Inspiratory crackles

DX: CXR shows fibrosis

  • CT chest: diffuse patchy fibrosis with pleural based honeycombing
  • PFTs = restrictive (decreased lung volume, normal/increased FEV1/FVC ratio)

Tx: Few effective treatment options other than a lung transplant

  • corticosteroids, O2
Pneumoconiosis Any fibrosis of the lung tissues with a known cause - usually from prolonged environmental or occupational contact

  • Coal worker’s: coal mining; complication = progressive massive fibrosis
    • CXR: small nodular opacities in upper lung fields
  • Silicosis: mining, sandblasting, stone, quarry work; increased risk TB and progression to massive fibrosis
    • CXR: small rounded opacities throughout the lung, hilar lymph nodes may be calcified - "eggshell" calcifications
  • Asbestos: insulation, demolition, shipbuilding, construction; complication = mesothelioma
    • CXR: interstitial fibrosis, thickened pleura, calcified plaques appear on diaphragms or lateral chest wall
  • Berylliosis: high tech field, nuclear power, ceramics, aerospace, electrical plants, foundries; requires chronic steroids
    • CXR: diffuse infiltrates and hilar adenopathy

S/SX: SOB + nonproductive cough + chronic hypoxia, cor pulmonale

DX: CXR: interstitial fibrosis

  • PFTs = reduced lung volumes - restrictive dysfunction and reduced diffusing capacity

Tx: primarily supportive ⇒ oxygen, vaccinations (pneumococcal, flu) and rehab

  • Steroids to relieve chronic alveolitis
  • Smoking cessation = synergistically linked to lung cancer
 Sarcoidosis Chronic autoimmune inflammatory disease in which small nodules (granulomas) develop in lungs, lymph nodes, and other organs

↑ ACE levels + Bilateral hilar adenopathy


  • Pulmonary manifestations (most common), erythema nodosum, parotid gland enlargement
  • Chest radiograph: Bilateral hilar lymphadenopathy. Reticular infiltrates.
  • Lupus pernio (chronic, violaceous, raise plaques and nodules commonly found on cheeks, nose, eyes) = pathognomonic for sarcoid and most specific physical exam finding
  • Biopsy: non-caseating granulomas

Treatment: Steroids = 90% respond to steroid

  • Methotrexate, other immunosuppressive meds
  • Serial PFTs to assess disease progression/guide treatment
  • ACE-I for periodic HTN

Prognosis depends on disease severity; spontaneous improvement common

Lesson Intro Video

Pulmonary hypertension (Prev Lesson)
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