Restrictive Lung Diseases Overview Flashcards

1
Q

What three things can cause muscle weakness - intercostal or diaphragmatic - which causes thoracic restriction due to causes outwith the lungs?

A

Myopathy
Neuropathy
Myelopathy

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2
Q

What do Myaesthenia gravis, Guillan Barre, Motor neurone disease and poliomyelitis all cause, which leads to thoracic restriction of teh lungs?

A

Muscle weakness

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3
Q

How can abdominal obesity/ascites cause thoracic restriction to the lungs?

A

Compression of thoracic contents

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4
Q

What does thoracic restriction due to causes out with the lungs result in?

A

Chronic alveolar under ventilation with low PaO2 (decreased SaO2) and raised PaCO2 and reduced lung volumes

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5
Q

Name 5 causes within the lungs that cause thoracic restriction?

A
  1. Acute DPLD - diffuse parenchymal lung disease
  2. Episodic DPLP, all of which may present acutely
  3. Chronic DPLD due to occupational or environmental agents
  4. Chronic DPLD with evidence of systemic disease
  5. Chronic DPLD with no evidence of systemic disease
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6
Q

What is lung parenchyma?

A

Alveolar walls/lumen

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7
Q

What is the pathophysiology of restrictive thoracic disease due to diseases within the lung i.e. DPLD?

A

Imparied alveolar gas exchange - alveolar barrier to O2 exchange, CO2 exchange umimparied as alveolar ventilation normal

Leads to decreased PaO2 (decreased SaO2) and normal PaCO2

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8
Q

What can cause DPLD?

A
Fluid in the alveolar air spaces
Consolidation of alveolar air spaces
Inflammatory infiltrate of alveolar walls (alveolitis)
Dust disease
Carcinomatosis
Eosinophilic
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9
Q

What two conditions can lead to fluid in the alveolar air sacs?

A

Cardiac pulmonary oedema (in alveolar walls and lumen)

Non-cardiac pulmonary oedema

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10
Q

What causes cardiac pulmonary oedema?

A

Raised pulmonary venoius pressure i.e. LVF

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11
Q

What causes non-cardiac pulmonary oedema?

A

Normal pulmonary venous pressure with leaky pulmonary capillaries - due to sepsis or trauma (ARDS aka shock lung) also due to altitude sickness

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12
Q

Give three causes of consolidation of alveolar air spaces?

A

Infective pneumonia
Infarction - PE/vasculitis
BOOP - Rheumatoid disease

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13
Q

Name an inflammatory infiltrate cause of alveolitis?

A

Granulomatous alveolitis

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14
Q

Give two granulomatous-alveolitis diseases?

A

Extrinsic allergic alveolitis

Sarcoidosis

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15
Q

What do Farmer’s lung and avian cause?

A

Extrinsic-Allergic-Alveolitis aka hypersensitivity pneumonitis- type III reaction

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16
Q

What is the name of a multi-system disease with clinical features such as lymphadenopathy, erythema nodosum, uveitis, myocarditis and neuropathy?

A

Sarcoidosis

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17
Q

What three drugs can induce alveolitis?

A

Amiodarone
Bleomycin
Methotrexate

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18
Q

What toxic gas fume can cause DPLD, alveolitis?

A

Chlorine

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19
Q

Give two features of fibrosing alveolitis?

A

Rheumatoid, cryptogenic

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20
Q

What classification of diseases do SLE, polyarteritis, Wegners, Churg-Straussand Bechet’s all come under?

A

Autoimmune (multi-systemic)

21
Q

What is another term for dust disease?

A

Pneumoconiosis

22
Q

What two subtypes are there to dust disease?

A

Fibrogenic and non-fibrogenic

23
Q

Name 2 fibrogenic dust diseases?

A

Asbestosis

Silicosis

24
Q

Name 3 non-fibrogenic dust-diseases?

A

Siderosis (iron)
Stanosis (Tin)
Baritosis (Barium)

25
Q

What is nitrofurantoin?

A

Eosinophilic drug response

26
Q

What is Aspergillosis?

A

Fungal eosinophilic response

27
Q

What is Toxocara, Ascaris and Filaria?

A

Eosinophilic parasite response

28
Q

What are Churg Strauss and Polyarteritis?

A

Autoimmune eosinophilic responses

29
Q

What does pulmonary fibrosis occur as an end stage response to?

A

Chronic inflammation

30
Q

Give 6 clinical features of DPLD?

A
  1. Breathless on exertion
  2. Cough but no wheeze
  3. Finger clubbing
  4. Inspiratory lung crackles
  5. Central cyanosis (if hypozaemic)
  6. Pulmonary fibrosis
31
Q

What can be said about the lung volumes in the diagnosis of DPLD?

A

Reduced lung volumes:
Decreased FEV1
Decreased FVC normal ration > 75%
Peak flow is normal

32
Q

What condition has reduced gas diffusion (DLCO) and arterial oxygen desaturation (decreased PaO2 and decreased SaO2)?

A

DPLD

33
Q

What might be raised in sarcoidosis and so be used for diagnosis?

A

Serum ACE and Ca

34
Q

In some DPLD what antibodies might you look for?

A

Avian, fungal, auto-antibodies (rheumatoid, anti-nuclear)

35
Q

In DPLD will there be bilateral diffuse alveolar infiltrates on CXR?

A

Yes

36
Q

In the diagnosis of DPLD, why would you do an echocardiogram?

A

To exclude LVF

37
Q

What investigation can differentiate between inflammatory ground glass and fibrotic nodular component of alveolar infiltrates?

A

High resolution CT

38
Q

In the diagnosis of DPLD - why mighy you do bronchoalveolar lavage or induced sputum?

A

To exclude pneumocysits and TB

39
Q

If there is ground glass inflammation on HRCT, what would you treat with?

A

Immunosuppressives

40
Q

What is the first line treatment of DPLD?

A

Systemic corticosteroids i.e. oral prednisolone

41
Q

Can you use inhaled corticosteroids to treat DPLD?

A

No

42
Q

What is the second line treatment for DPLD?

A

Oral azathioprine [steroid sparing]

43
Q

Name an anti-fibrotic agent?

A

Pirfenidone

44
Q

What would you use anti-fibrotic agents for?

A

Idiopathic pulmonary fibrosis

45
Q

What would you use anti-oxidant agents for?

A

Acetylcysteine for Idiopathic Pulmonary Fibrosis

46
Q

In what disease would you get bilateral hilar lymphadenopathy and lung infiltrates on CXR?

A

Sarcoidosis

47
Q

What type of granulomas does sarcoidosis have?

A

Non caseating ggranuloma

48
Q

What three skeletal related conditions can lead to thoracic restriction due to causes out with lungs?

A
  1. Thoracic kyphoscoliosis
  2. Ankylosing spondylitis
  3. Rib trauma