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The respiratory system > Restrictive lung disease > Flashcards

Restrictive lung disease Flashcards

1
Q

What is intrinsic restrictive lung disease?

A
  • disease of the lung itself

- results in impaired gas exchange

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2
Q

How does the FVC change in restrictive lung disease?

A
  • it decreases
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3
Q

What is the FEV1/FVC ratio in restrictive lung disease?

A
  • greater than 70
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4
Q

How does the DLCO change in restrictive lung disease?

A
  • decreased

- less than 80% predicted

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5
Q

How is gas exchange impaired in intrinsic restrictive lung disease?

A
  • alveolar wall becomes thicker
  • impaired exchange of O2
  • normal exchange of CO2
  • decreased PaO2 and normal PaCO2
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6
Q

what does the diffusion capacity of carbon monoxide measure (DLCO)?

A
  • measures the gas diffusion across the alveolar - arteriolar barrier
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7
Q

What is extrinsic restrictive lung disease?

A
  • (damage to) structures outside of the lungs have an effect on lung function
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8
Q

What are some of the causes of extrinsic restrictive lung disease?

A
  • thoracic/extra thoracic: e.g. obesity, kypho - scoliosis
  • neuromuscular disorders
  • pleural disease
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9
Q

What does Hypoventilation cause?

A
  • rise in paCO2 and a reduced paO2
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10
Q

How does obesity/kypho-scoliosis effect lung function?

A
  • They reduce chest wall compliance
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11
Q

How do neuromuscular conditions effect lung function?

A
  • cause reduced power of muscles involved in respiration
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12
Q

How do patients with restrictive lung disease present?

A
  • progressive shortness of breath
  • +/- dry cough
  • history is key: drugs, smoking, pre existing neuromuscular disorder, CO2 retention symptoms
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13
Q

What may be found on clinical examination on a patient with restrictive lung disease?

A
  • finger clubbing
  • obese/kyphosis scoliosis
  • fibrotic crepitations
  • clinical signs of pleural effusion or ascites
  • cyanosis
  • CO2 retention
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14
Q

How is restrictive lung disease treated?

A
  • lifestyle change (weight loss)
  • removal of trigger factor
  • medical therapy
  • procedures: intercostal drainage ect
  • surgery
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15
Q

Describe the medical therapy that may be used in the treatment of restrictive lung disease?

A
  • anti- inflammatory/ immunosuppressive drugs (steroids)
  • anti fibrotic drugs
  • treating neuromuscular disease
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16
Q

What surgery options may be available to a patient with restrictive lung disease?

A
  • corrective spinal surgery
  • decortication for chronic empyema
  • diaphragmatic plication
  • lung transplant (fibrosis)
17
Q

Describe the supportive treatments available for patients with restrictive lung disease?

A
  • CPAP - for OSA
  • non invasive ventilation
  • oxygen (caution re sensitivity in CO2 retainers)
18
Q

What is the pulmonary interstitium?

A
  • type 1 and 2 alveolar lining cells

- thin elastin rich connective component containing capillary blood vessels

19
Q

What are the stages of interstitial lung disease?

A
  • early stage: alveolitis, injury with inflammatory cell infiltration
  • late stage: characterised by fibrosis
20
Q

What are the causes of interstitial lung disease?

A
  • environmental: minerals, drugs, radiation
  • hypersensitivity: mouldy hay, avian proteins
  • unknown: connective tissue disease, IPF
21
Q

How is interstitial lung disease diagnosed?

A
  • usually though a biopsy
22
Q

what biopsies are used in the diagnosis of interstitial lung disease?

A
  • trans bronchial biopsy

- thoracoscopic biopsy: more invasive but more reliable

23
Q

What are some examples of chronic interstitial disease?

A
  • idiopathic pulmonary fibrosis
  • sarcoidosis
  • hypersensitivity pneumonitis
  • pneumoconiosis
  • connective tissue disease
24
Q

describe IPF?

A
  • progressive interstitial fibrosis of unknown cause
  • variable associated inflammation
  • associated with finger clubbing
25
Q

Describe the pathology of IPF?

A
  • sub pleural and basal fibrosis
  • inflammatory component variable
  • terminally lung structure replaced by dilated spaces surrounded by fibrous walls
26
Q

Describe hypersensitive pneumonitis?

A
  • chronic inflammatory disease
  • affects small airways and interstitium with occasional granulomas
  • allergic origin: can by type III or type IV
27
Q

what are the causes of Hypersensitive pneumonitis?

A
  • thermophilic bacteria: famers lung
  • Avian proteins: bird fanciers lung
  • fungi: malt workers lung
28
Q

What is sarcoidosis?

A
  • multi system non caseating granulomatous disorder of unknown cause
  • pulmonary involvement is common
  • most cases it is mild and self limiting
29
Q

Describe some other manifestations of sarcoidosis?

A
  • uveitis (inflammation of iris)
  • erythema nodosum
  • lymphadenopathy
  • hypercalcaemia
30
Q

Describe cases of pulmonary involvement in connective tissue disease?

A
  • interstitial fibrosis (milder than IPF)
  • pleural effusions
  • rheumatoid nodules
31
Q

What is pneumoconiosis?

A
  • lung disease caused by mineral dust exposure
  • asbestosis
  • coal workers lung
  • silicosis
32
Q

What diseases can be related to asbestos?

A
  • parietal pleural plaques
  • interstitial fibrosis
  • bronchial carcinoma
  • mesothelioma
33
Q

what can occur in acute sarcoidosis?

A
  • erythema nodosum
  • bilateral hilar lymphadenopathy
  • arthritis
  • uveitis, parotitis
  • fever
34
Q

What can occur in chronic sarcoidosis?

A
  • lung infiltrates
  • skin infiltrations
  • peripheral lymphadenopathy
  • hypercalcaemia
  • other organs
35
Q

describe acute hypersensitivity pneumonitis?

A
  • cough, breathlessness, fever, myalgia
  • symptoms occur several hours after exposure
  • widespread pulmonary infiltrates on CXR
36
Q

Describe chronic hypersensitive pneumonitis?

A
  • repeated low dose antigen exposure over time
  • progressive breathlessness and cough
  • pulmonary fibrosis in upper lobes
37
Q

Describe the treatment of a patient with hypersensitive pneumonitis?

A
  • oxygen
  • oral steroid
  • antigen avoidance
38
Q

Describe the clinical presentation of a patient with IPF?

A
  • progressive breathlessness and dry cough
  • clubbing, bilateral fine inspiratory crackles
  • CXR shows bilateral infiltrates
  • CT shows reticulonodular fibrosis worse at lung bases and periphery and traction brocheicstasis

The respiratory system (13 decks)

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