Restrictive lung disease

Question 1

What is intrinsic restrictive lung disease?

Answer 1

• disease of the lung itself

- results in impaired gas exchange

Question 2

How does the FVC change in restrictive lung disease?

Answer 2

• it decreases

Question 3

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

Answer 3

• greater than 70

Question 4

How does the DLCO change in restrictive lung disease?

Answer 4

• decreased

- less than 80% predicted

Question 5

How is gas exchange impaired in intrinsic restrictive lung disease?

Answer 5

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

Question 6

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

Answer 6

• measures the gas diffusion across the alveolar - arteriolar barrier

Question 7

What is extrinsic restrictive lung disease?

Answer 7

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

Question 8

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

Answer 8

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

Question 9

What does Hypoventilation cause?

Answer 9

• rise in paCO2 and a reduced paO2

Question 10

How does obesity/kypho-scoliosis effect lung function?

Answer 10

• They reduce chest wall compliance

Question 11

How do neuromuscular conditions effect lung function?

Answer 11

• cause reduced power of muscles involved in respiration

Question 12

How do patients with restrictive lung disease present?

Answer 12

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

Question 13

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

Answer 13

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

Question 14

How is restrictive lung disease treated?

Answer 14

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

Question 15

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

Answer 15

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

Question 16

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

Answer 16

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

Question 17

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

Answer 17

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

Question 18

What is the pulmonary interstitium?

Answer 18

• type 1 and 2 alveolar lining cells

- thin elastin rich connective component containing capillary blood vessels

Question 19

What are the stages of interstitial lung disease?

Answer 19

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

Question 20

What are the causes of interstitial lung disease?

Answer 20

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

Question 21

How is interstitial lung disease diagnosed?

Answer 21

• usually though a biopsy

Question 22

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

Answer 22

• trans bronchial biopsy

- thoracoscopic biopsy: more invasive but more reliable

Question 23

What are some examples of chronic interstitial disease?

Answer 23

• idiopathic pulmonary fibrosis
• sarcoidosis
• hypersensitivity pneumonitis
• pneumoconiosis
• connective tissue disease

Question 24

describe IPF?

Answer 24

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

Question 25

Describe the pathology of IPF?

Answer 25

- sub pleural and basal fibrosis - inflammatory component variable - terminally lung structure replaced by dilated spaces surrounded by fibrous walls

Question 26

Describe hypersensitive pneumonitis?

Answer 26

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

Question 27

what are the causes of Hypersensitive pneumonitis?

Answer 27

- thermophilic bacteria: famers lung - Avian proteins: bird fanciers lung - fungi: malt workers lung

Question 28

What is sarcoidosis?

Answer 28

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

Question 29

Describe some other manifestations of sarcoidosis?

Answer 29

- uveitis (inflammation of iris) - erythema nodosum - lymphadenopathy - hypercalcaemia

Question 30

Describe cases of pulmonary involvement in connective tissue disease?

Answer 30

- interstitial fibrosis (milder than IPF) - pleural effusions - rheumatoid nodules

Question 31

What is pneumoconiosis?

Answer 31

- lung disease caused by mineral dust exposure - asbestosis - coal workers lung - silicosis

Question 32

What diseases can be related to asbestos?

Answer 32

- parietal pleural plaques - interstitial fibrosis - bronchial carcinoma - mesothelioma

Question 33

what can occur in acute sarcoidosis?

Answer 33

- erythema nodosum - bilateral hilar lymphadenopathy - arthritis - uveitis, parotitis - fever

Question 34

What can occur in chronic sarcoidosis?

Answer 34

- lung infiltrates - skin infiltrations - peripheral lymphadenopathy - hypercalcaemia - other organs

Question 35

describe acute hypersensitivity pneumonitis?

Answer 35

- cough, breathlessness, fever, myalgia - symptoms occur several hours after exposure - widespread pulmonary infiltrates on CXR

Question 36

Describe chronic hypersensitive pneumonitis?

Answer 36

- repeated low dose antigen exposure over time - progressive breathlessness and cough - pulmonary fibrosis in upper lobes

Question 37

Describe the treatment of a patient with hypersensitive pneumonitis?

Answer 37

- oxygen - oral steroid - antigen avoidance

Question 38

Describe the clinical presentation of a patient with IPF?

Answer 38

- 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