PANCE Blueprint Pulmonary (10%)

Restrictive Pulmonary Disease (PEARLS)

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

osmosis Osmosis
Idiopathic pulmonary fibrosis
Patient will present as → a 55-year-old female who is a current smoker presents with a 9-month history of respiratory symptoms, including dyspnea on exertion, thoracic pain, and dry cough, which were preceded by a pulmonary infection. On auscultation, you hear inspiratory crackles. Pulmonary function tests (PFTs) show only mild impairment of vital capacity with decreased lung volume and a normal to increased FEV1/FVC ratio.

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
Patient will present as → a 53-year-old man who presents to the office complaining of progressive dyspnea over the past few years. History reveals that he has worked in construction for the past 20 years demolishing and refurbishing old buildings. He rarely uses any protective breathing equipment. Physical examination demonstrates an afebrile man in mild respiratory distress with inspiratory crackles. The chest x-ray reveals a reticular linear pattern with basilar predominanceopacities, and honeycombing. (asbestosis)

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
Patient will present as → a 30-year-old African American female with a cough, fever, and generalized body aches. You order a CXR which shows bilateral hilar adenopathy.

Chronic autoimmune inflammatory disease in which small nodules (granulomas) develop in lungs, lymph nodes, and other organs

↑ ACE levels + ↑ calcium levels + bilateral hilar adenopathy

Presentation:

  • 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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