Approching Patients W/ Restrictive Respiratory Diseases Flashcards
Restrictive lung diseases general definitions
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
HRCT honeycombing
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)
Idiopathic pulmonary fibrosis (IPF)
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
What is usually the main test needed for suspicion of RLDs other than PFT testing?
Lung tissue biopsy
Medications that can cause Interstital lung diseases
Methrotextrate
Azothioprine
Rituximab
Nitrofurantoin
Anti-TNFs
Amiodarone
Bleomycin
Pneumoconiosis
Inorganic or organic solvents/particles that get inhaled in the lungs and cause Intersitial lung diseases
What systemic diseases can cause interstital lung diseases?
Connective tissue disorders
SLE
Most types of chronic vasculitis
Cancer (specifically lung)
Sarcoidosis
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
Scleroderma (systemic sclerosis) (SSc)
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