Approching Patients W/ Restrictive Respiratory Diseases

Question 1

Restrictive lung diseases general definitions

Answer 1

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

Question 2

HRCT honeycombing

Answer 2

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)

Question 3

Idiopathic pulmonary fibrosis (IPF)

Answer 3

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

Question 4

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

Answer 4

Lung tissue biopsy

Question 5

Medications that can cause Interstital lung diseases

Answer 5

Methrotextrate

Azothioprine

Rituximab

Nitrofurantoin

Anti-TNFs

Amiodarone

Bleomycin

Question 6

Pneumoconiosis

Answer 6

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

Question 7

What systemic diseases can cause interstital lung diseases?

Answer 7

Connective tissue disorders

SLE

Most types of chronic vasculitis

Cancer (specifically lung)

Question 8

Sarcoidosis

Answer 8

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

Question 9

Scleroderma (systemic sclerosis) (SSc)

Answer 9

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