Respiratory - restrictive disease

Question 1

What are the types of restrictive diseases (2)

Answer 1

interstitial
Extra-pulmonary

Question 2

What are the symptoms of interstitial lung disease (5)

Answer 2

progressive dyspnoea
Dry cough
Headache
Confusion
Lethargy

Question 3

What causes headache, confusion, and lethargy in interstitial disease

Answer 3

Carbon dioxide retention

Question 4

What are the signs of CO2 retention due to interstitial disease (6)

Answer 4

flushed skin
Bounding pulse
Rapid breathing
Premature heartbeats
Muscle twitches
Flapping tremor

Question 5

What investigations are used for restrictive lung disease (4)

Answer 5

pulmonary function tests
Imaging (CXR, CT, and USS)
ABG
Bloods

Question 6

What results would be obtained in PFTs in restrictive lung disease

Answer 6

reduced FEV1 and FVC
Normal ratio

Question 7

What lifestyle treatments can be used for restrictive lung disease (2)

Answer 7

weight loss
Pulmonary rehab

Question 8

What medical therapies can be used to treat restrictive lung disease (3)

Answer 8

Anti-inflammatory drugs/Immunosuppressive drugs
Anti-fibrotic drugs
Treating neuromuscular disease

Question 9

What procedures can be used to treat restrictive lung disease (2)

Answer 9

Intercostal drainage
Ascitic drainage

Question 10

What surgeries can be used to treat restrictive lung disease (4)

Answer 10

corrective spinal surgery
Decorticating
Diaphragmatic privation
Lung transplant

Question 11

What methods of supportive treatment can be used in restrictive lung disease (3)

Answer 11

CPAP
Non-invasive ventilation
Oxygen

Question 12

Describe pulmonary interstitium (2)

Answer 12

connective tissue making up alveolar walls
Contains collagen, elastin, and capillaries

Question 13

General description of interstitial disease

Answer 13

disorders associated with inflammation and fibrosis of the interstitium of the lungs

Question 14

What are the stages of interstitial lung disease associated with (2)

Answer 14

early - alveolitis
Later - fibrosis

Question 15

What functional abnormalities are caused by interstitial lung disease (3)

Answer 15

reduction in diffusion capacity
Reduction in lung volume
Reduction in lung compliance

Question 16

What are the categories of interstitial disease (5)

Answer 16

fibrosing
Granulomatous
Eosinophilic
Smoking related
Other

Question 17

What are examples of fibrosing interstitial lung disease (3)

Answer 17

usual interstitial pneumonia
Connective tissue associated
Pneumoconiosis

Question 18

What are examples of granulomatous interstitial diseases (2)

Answer 18

Sarcoiditis
Hypersensitivty pneumonitis

Question 19

What are the causes of interstitial disease (3)

Answer 19

Environmental
Hypersensitivity
Idiopathic

Question 20

What are the symptoms of interstitial disease (2)

Answer 20

dry cough
Breathlessness

Question 21

Describe usually interstitial pneumonia (3)

Answer 21

progressive pulmonary fibrosis
Associated with patchy interstitial fibrosis, early lesions, and late lesions
Leads to respiratory failure

Question 22

What regions of the lobe are affected in usual interstitial pneumonia

Answer 22

basal regions of lower lobes

Question 23

What connective tissue disease are associated with interstitial disease (3)

Answer 23

rheumatoid arthritis
SLE
Systemic sclerosis

Question 24

Describe components of connective tissue disease associated interstitial disease (3)

Answer 24

Interstitial fibrosis
Pleural effusion
Rheumatoid nodules (in rheumatoid arthritis)

Question 25

What is pneumoconiosis caused by

Answer 25

reaction to inhaled particles

Question 26

What are examples of pneumoconiosis (3)

Answer 26

coal worker’s pneumoconiosis Silicosis Asbestosis

Question 27

Describe Pathophysiology of silicosis (4/5)

Answer 27

particles are inhaled then phagocytosed by macrophages An inflammatory response is induced Hard collagenous scars form (Progressive massive fibrosis may develop) Egg-shell calcification hilar nodes occurs

Question 28

Describe Pathophysiology of asbestosis (3)

Answer 28

inhaled particles are phagocytosed An inflammatory response is stimulated Parenchymal interstitial fibrosis occurs

Question 29

What complications is asbestosis linked to (5)

Answer 29

pleural plaques Recurrent pleural effusions Interstitial fibrosis Lung carcinoma Mesothelioma

Question 30

Describe sarcoidosis (3)

Answer 30

A systemic granulomatous disease Linked to type 4 Hypersensitivity With granulomas distributed around bronchi and blood vessels along lymphatics

Question 31

Describe granulomas in sarcoidosis (2)

Answer 31

well formed Non-caseating

Question 32

How can sarcoidosis be diagnosed (3)

Answer 32

CXR and CT showing peripheral nodular infiltrate Tissue biopsy showing non-caseating granulomas Raised ACE, calcium, and inflammatory markers in blood test

Question 33

how is chronic sarcoidosis treated (3)

Answer 33

monitoring Oral steroids Immunosuppression

Question 34

When would acute sarcoidosis be treated

Answer 34

if a vital organ is affected

Question 35

What type of Hypersensitivity is involved in hypersensitivity pneumonitis

Answer 35

type 3

Question 36

What are the signs of acute hypersensitivity pneumonitis (7)

Answer 36

cough Breathlessness Fever Myalgia Pyrexia Crackles hypoxia

Question 37

What are the signs of chronic hypersensitivity pneumonitis (4)

Answer 37

crackles Finger clubbing Pulmonary fibrosis Restrictive defect

Question 38

which part of the lungs is affected by hypersensitivity pneumonitis

Answer 38

Upper zones

Question 39

Describe histology of hypersensitivity pneumonitis (4)

Answer 39

Airway entry disease Interstitial inflammatory infiltrate Loosely formed, non-caseating granulomas Interstitial fibrosis

Question 40

How is hypersensitivity pneumonitis diagnosed (3)

Answer 40

history of exposure Precipitants Lung biopsies

Question 41

How is hypersensitivity pneumonitis treated (5)

Answer 41

oxygen Steroids Antigen avoidance Immunosuppression (if chronic) Anti-fibrotic therapy (if progressive fibrosis)

Question 42

Describe idiopathic pulmonary fibrosis (2)

Answer 42

a non-inflammatory condition Linked to an imbalance in the fibrotic repair system

Question 43

Describe what happens in idiopathic pulmonary fibrosis (2)

Answer 43

fibrosis of alveolar walls occurs (fibroblastic foci) Destruction of architecture (honeycombing)

Question 44

Describe the clinical presentation of idiopathic pulmonary fibrosis (6)

Answer 44

Progressive breathlessness Dry cough Clubbing Bilateral fine inspiratory crackles Restrictive defect Bilateral infiltrates

Question 45

What shows on a CT scan in cases of idiopathic pulmonary fibrosis (3)

Answer 45

reticulonodular fibrotic shadowing Traction bronchiectasis Honeycombing cystic changes

Question 46

How can idiopathic pulmonary fibrosis be treated (3)

Answer 46

anti-fibrotic drugs Oxygen (if hypoxic) Lung transplant