Restrictive Thoracic Disease Flashcards

1
Q

What are causes of thoracic restriction outwith the lungs?

A
  • Skeletal: vertebrae (e.g. kyphoscoliosis), ankylosing spondylitis, ribs (e.g. multiple rib fracture)
  • Muscle weakness: intercostal or diaphragmatic (due to myopathy, neuropathy, myelopathy) e.g. Myasthenia Gravis, Guillian Barre, Motor Neurone Disease, Poliomyelitis
  • Abdominal obesity/ascites - compression of diaphragm into thoracic cage, cannot contract and descend as effectively
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2
Q

What is myopathy?

A

Disease of the muscle

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3
Q

What is neuropathy?

A

Disease of the nerves

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4
Q

What is myelopathy?

A

Disease of the spinal cord

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5
Q

What are examples of diseases that cause muscle weakness?

A
  • Myaesthenia Gravis
  • Guillan Barre
  • Motor neurone disease
  • Poliomyelitis
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6
Q

What is ascites?

A

Build up of fluid in abdominal cavity

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7
Q

What does thoracic restriction result in?

A
  • Chronic alveolar hypoventilation with decreased PaO2 (hypoxia) and increased PaCO2 (hypercapnia)
  • Reduced lung volumes
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8
Q

What are the causes of thoracic restriction originating from within the lungs?

A
  • Acute DPLD (interstitial lung disease)
  • Episodic DPLD
  • Chronic DPLD due to occupational or environmental agents/drugs
  • Chronic DLPD with evidence of systemic disease
  • Chronic DLPD with no evidence of systemic disease
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9
Q

What is DPLD?

A

Interstitial lung disease

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10
Q

What is lung interstition?

A

Area between alveoli

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11
Q

What is restrictive thoracic disease?

A

Occurs due to disease within the lungs

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12
Q

What are examples of diseases that cause thoracic restriction?

A
  • Disease of alveolar structures (walls/lumen) e.g. DPLD

* Pathophysiology - impaired exchange of O2, but not CO2

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13
Q

What does Alveolar-Arteriolar barrier determine?

A

How effectively oxygen is transferred across alveolar epithelium into red blood cells where it binds to haemoglobin

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14
Q

Why is CO2 exchange unimpaired due to pathophysiology?

A
  • Alveolar ventilation is normal

* CO2 is very soluble and blown off (normal PaCO2)

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15
Q

What are causes of DPLD?

A
  • Fluid in the alveolar air spaces
  • Consolidation of alveolar air spaces
  • Inflammatory infiltrate of alveolar walls (i.e. alveolitis)
  • Drug-induced alveolitis
  • Toxic gas/fumes
  • Pulmonary fibrosis
  • Autoimmune (multi-system)
  • Dust-disease (pneumoconiosis)
  • Carcinomatosis
  • Eosinophilic allergic response
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16
Q

What is ARDS?

A

Adult respiratory distress syndrome (shock lung) caused by leaky pulmonary capillaries

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17
Q

What are causes of consolidation of alveolar air spaces?

A
  • Infective pneumonia (most common cause)
  • Pulmonary infarction (pulmonary emboli)
  • BOOP (bronchiolitis obliterans organising pneumonia)
18
Q

What causes fluid to build up in alveolar air spaces?

A
  • Cardiac Po oedema (in alveolar walls and lumen) due to raised Po venous pressure e.g. due to left ventricular failure
  • Non cardiac Po oedema - normal Po venous pressure with leaky Po capillaries due to sepsis or trauma
19
Q

What causes BOOP?

A
  • Rheumatoid disease
  • Drugs
  • Cryptogenic (unknown cause)
20
Q

What are the 2 causes of granulomatous alveolitis?

A
  • Extrinsic allergic alveolitis

* Sarcoidosis

21
Q

What is extrinsic allergic alveolitis?

A

Hypersensitivity type 3 reaction due to extrinsic antigen (not allergen as not mediated by IgE)

22
Q

What are examples extrinsic allergic alveolitis?

A
  • Farmer’s lung

* Avian

23
Q

What inflammation is caused by extrinsic allergic alveolitis and sarcoidosis?

A

No eosinophilic inflammation - gives lymphocytic inflammation, causing formation of granulomas

24
Q

What is sarcoidosis?

A

A disease that results in granulomatic lymphocytic inflammation

25
Q

What is the cause of sarcoidosis?

A

No known cause

26
Q

What drugs can induce alveolitis?

A
  • Amiodarone
  • Bleomycin, Methotrexate
  • Gold
27
Q

What toxic gas can cause DPLD?

A

Chlorine

28
Q

What are examples of autoimmune diseases that cause DPLD?

A
  • Systemic lupus erythematosus (SLE)
  • Polyarteritis
  • Wegeners
  • Churg-Strauss
  • Bechet’s
29
Q

What is pneumoconiosis?

A

Dust-disease - usually associated with fibrosis

30
Q

What are the 2 types of pneumoconiosis?

A
  • Fibrogenic

* Non-fibrogenic

31
Q

What are causes of fibrogenic pneumoconiosis?

A
  • Asbestosis

* Silicosis

32
Q

What are causes of non-fibrogenic pneumoconiosis?

A
  • Siderosis (Iron)
  • Stanosis (Tin)
  • Baritosis (Barium)
33
Q

What are glandular adenocarcinomas that metastasise via blood and lymphatics to the lungs?

A
  • Bronchial adenocarcinoma
  • Breast adenocarcinoma
  • Prostate adenocarcinoma
  • Colon adenocarcinoma
  • Stomach adenocarcinoma
34
Q

What are clinical signs of DPLD?

A
  • Breathless on exertion
  • Cough but no wheeze (wheeze indicated airway obstruction)
  • Finger clubbing
  • Inspiratory lung crackles
  • Central cyanosis (if hypoxaemic)
  • Pulmonary fibrosis as end stage response to chronic inflammation
35
Q

How is DPLD diagnosed?

A
  • History e.g. occupation, drugs, pets
  • Reduced lung volumes - decreased FEV1 and FVC, FEV1/FVC ratio normal >75%
  • peak flow normal
  • Reduced gas diffusion (DLCO)
  • Arterial oxygen desaturation (PaO2, SaO2)
  • Antibodies (avian, fungal)
  • ACE and Ca raised in sarcoidosis
  • CXR
  • Echocardiogram to exclude heart failure and diagnose secondary pulmonary hypertension
  • High resolution CT scan (can be used to stage disease)
  • Sputum/bronchoalveolar lavage
  • Transbronchial or thorascopic lung biopsy
36
Q

What is PaO2?

A

Partial pressure of oxygen in arterial blood

37
Q

What is SaO2?

A

The volume of oxygen bound to haemoglobin in arterial blood

38
Q

What is the treatment for DPLD?

A
  • Remove trigger factor
  • Treat reversible alveolitis e.g. immuno-suppressives
  • Systemic steroids - ICS not effective, treated with Azathioprone
  • IPF treated with anti-fibrotic agents e.g. pirfenidone, nintendanib
  • Oxygen if hypoxaemic
  • Lung transplantation (last resort)
39
Q

Why are inhaled corticosteroids not effective in treating interstitial lung disease?

A

Not a high enough concentration in distal lung

40
Q

Do anti-fibrotic agents destroy collagen?

A

No, they only prevent further deposition of collagen

41
Q

Why are pirfenidone and nintendanib not first line treatments?

A

Horrible side effects

42
Q

What are clinical signs of sarcoidosis?

A
  • Erythema
  • Bilateral hilar lymphadenopathy (viewed on CXR)
  • Lung infiltrates (viewed on CXR)