PANCE Blueprint Pulmonary (10%)

• 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

• 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

↑ ACE 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

• Pulmonary fibrosis = leading cause of death