Approching Patients W/ Restrictive Respiratory Diseases Flashcards

1
Q

Restrictive lung diseases general definitions

A

Expansion of the lungs are restricted
- also called “ lung parenchyma diseases or/ lung interstitum diseases)”

In clues restriction in the following also

  • chest wall
  • pleural linings
  • neuromuscular junctions

Often shows diffuse fibrosis/ collagen buildup on electron micrographs or microscopy

Increases radial traction

1) causes decreases in
- FVC
- DLCO
- TLC

2) increased or normal FEV1/FVC ratio
- because FVC is decreasing more than FEV1

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2
Q

HRCT honeycombing

A

Found in traction bronchiectasis patients

High-resolution computed tomography (HRCT) image will show retracted/nodule lung segments w/ widened bronchiole diameter that is aechoic
- lungs look like a honey comb (kinda)

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3
Q

Idiopathic pulmonary fibrosis (IPF)

A

Idiopathic causes

  • although 1/3 is genetic
  • most often seen in elderly

Symptoms/signs

  • insidious onset of dyspnea, dry cough
  • bilateral dry rales on auscultation
  • classic restrictive patterns seen on PFTs
  • honey combing on HRTC
  • Mortality rates are 50% in 3-5 yrs of diagnosis*

Treatment:

  • supportive oxygen
  • lung transplants
  • anti-fibrotic agents
  • immune modulators
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4
Q

What is usually the main test needed for suspicion of RLDs other than PFT testing?

A

Lung tissue biopsy

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5
Q

Medications that can cause Interstital lung diseases

A

Methrotextrate

Azothioprine

Rituximab

Nitrofurantoin

Anti-TNFs

Amiodarone

Bleomycin

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6
Q

Pneumoconiosis

A

Inorganic or organic solvents/particles that get inhaled in the lungs and cause Intersitial lung diseases

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7
Q

What systemic diseases can cause interstital lung diseases?

A

Connective tissue disorders

SLE

Most types of chronic vasculitis

Cancer (specifically lung)

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8
Q

Sarcoidosis

A

Abnormal development of clusters of inflammatory cells and/or antibody antigen complexes that get deposited throughout the body
- look like granulomas

Is a restrictive lung diseases when it affects the lungs

Most commonly affects lung but can affect the following:

  • skin
  • liver
  • eyes

Always produces hilar adenopathy

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9
Q

Scleroderma (systemic sclerosis) (SSc)

A

Restrictive lung disease that occurs in up to 50% of diffuse cases

Anti inflammatory disease that is produced via anti-Scl-70 antibody overproduction
- affects skin, esophagus pulmonary and cardiac tissues causing sclerosis of the affected tissues

Treatment = immunosupression therapies

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