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restrictive lung disease Flashcards

1
Q

define restrictive lung disease

A

decrease in total lung capacity (FVC + residual volume – max volume in lungs)

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2
Q

what are the 2 categories of restrictive lung disease?

A

intrinsic

extrinsic

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3
Q

what are intrinsic lung diseases?

A

Affecting the lung parenchyma – driven by inflammation that leads to lung scarring

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4
Q

what are extrinsic lung diseases?

A

Affecting the pleura, chest wall or neuromuscular apparatus

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5
Q

what % of interstitial lung diseases are of known origin? name some causes

A

35% of ILDs are DPLD of known cause e.g. pneumoconiosis e.g. asbestosis

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6
Q

what % of ILDs are idiopathic?

A

65%

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7
Q

name the major ILDs?

A

IPF, Sarcoidosis, pneumoconiosis

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8
Q

what is the aetiology of IPF?

A

unknown

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9
Q

what are risk factors for IPF?

A

smoking, environmental exposures, chronic viral infections, abnormal acid reflux and family history of the disease

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10
Q

how does IPF affect the lungs?

A

Results in scarring/honeycombing in lung –> restricts breathing and oxygen exchange

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11
Q

how can repetitive microinjuries damage the epithelium in IPF?

A 
causes o	Epithelial cell senescence
o	Epithelial apoptosis
o	Epithelial proliferation
o	Epithelial activation
o	Epithelial-mesenchymal transition
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12
Q

what is the proposed pathology of IPF?

A
  • environmental factors lead to repetitive microinjuries
  • Repetitive microinjuries can also cause the release of chemicals e.g. growth factors, developmental signals, cytokines, chemokines etc.  fibroblast migration and proliferation + differentiation to myofibroblasts and ECM accumulation
    • Fibroblast proliferation leads to scarring – form plaques which reduce ability of lungs to expand
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13
Q

what alveolar damage occurs in IPF?

A
  • IPF results in traction bronchiectasis (dilation of the bronchi)
  • Alveolar remodelling
  • Parenchymal fibrosis
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14
Q

what are the symptoms of IPF?

A
  • Dry cough
  • Exertional dyspnea
  • Clubbing
  • Breathlessness
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15
Q

how do you diagnose IPF?

A 
•	Medical history + symptoms
•	Physical examination with stethoscope
• Lung function tests - DLCO/TLCO
- high resolution CT
histology
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16
Q

what should you hear with a stethoscope in IPF?

A

Should hear; fine, high-pitched bibasilar inspiratory crackles (Velcro-like sounds)

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17
Q

what is the lung test for IPF?

A

DLCO/TLCO

18
Q

how is CO different to O2?

A

greater affinity for Hb than oxygen

19
Q

how does DLCO work?

A

• CO is inhaled at a particularly low concentration – measuring quantity of carbon monoxide (CO) transferred per minute from alveolar gas to red blood cells (mL/min/mm Hg)

20
Q

what are the units for the DLCO test?

A

o mL of CO transferred per minute for each mm Hg of pressure difference across the total available functioning lung gas exchange surface

21
Q

what is the equation for DLCO?

A

DLCO = Lung surface area available for gas exchange (Va) X rate of capillary blood CO uptake (Kco)

22
Q

what would affect your uptake ability in the DLCO test?

A

If you have fibrotic plaque and injury to the epithelial cell then your uptake ability is impaired

23
Q

what should a normal result be in the DLCO test?

A

• Anyone with DLCO >75% is considered normal – less than that implies restrictive lung disease

24
Q

what are the concerns with DLCO?

A

falsely reduced in individuals who fail to inspire to TLC + significant variation

25
Q

what does a CT show in IPF?

A

High resolution CT: reticular changes associated with traction bronchiectasis and honeycombing

26
Q

what is the treatment goal for IPF?

A

• Treatment goal is to improve symptoms + survival, preserve lung function and reduce adverse effects

27
Q

what are the 3 main drugs used for IPF treatment?

A

Pirfenidone, Nintedanib, Antacid therapy

28
Q

what type of drug is pirfenidone?

A

anti-fibrotic agent

29
Q

what effect does pirfenidone have?

A
  • ↓Physiological deterioration

* ↑Progression-free survival

30
Q

what are the adverse effects of pirfenidone?

A

GI, photosensitivity, anorexia, skin rash & liver toxicity

31
Q

what type of drug is nintedanib?

A

tyrosine kinase inhibitor

32
Q

what effect does nintedanib have?

A

decreased FVC decline

33
Q

what are the adverse effects of nintedanib?

A

diarrhoea, weight loss & liver toxicity

34
Q

what are the adverse effects of antacid therapy?

A

Infection, cognitive function & MI

35
Q

what are the non-pharmacotherapy treatments for IPF?

A
  • Pulmonary rehab: increases physical and psychological, increases walk distance and increases quality of life
  • Long term oxygen: patients with hypoxemia at rest
  • Short burst oxygen therapy: exertional hypoxemia
  • Lung transplant: moderate-severe disease
36
Q

what is sarcoidosis?

A

Acute + self-limiting inflammatory disease - immune system goes into overdrive
• Cause immune cells to clump together to form granulomas

37
Q

what part of the body does sarcoidosis affect?

A

Can affect almost any organ in the body – 90% of times it affects the lungs

38
Q

what is the cause of sarcoidosis?

A

cause isnt known

39
Q

what group of people does sarcoidosis affect?

A
  • Can affect people of any age but 70% of patients are 20 to 40
  • More common in African Americans and people of Northern European descent
40
Q

what organs does sarcoidosis affect and what symptoms does this cause?

A
  • Eye – dry eyes and blurry vision
  • Lymph nodes – enlarged
  • Lungs – hacking cough, coughing up blood
  • Heart – complications
  • Liver and spleen – enlargement
  • Joints – pain, arthritis, swelling of the knees
  • Skin – rashes, lupus pernio, erythema nodosum, skin lesions on the back, subcutaneous nodules
41
Q

what is the proposed pathology of sarcoidosis?

A
  • Aetiology unknown
  • Antigen phagocytosed by APC
  • APC present to T cells by HLA class 2 molecules
  • Formation of sarcoid granuloma
42
Q

how do you diagnose sarcoidosis?

A
  • Clinicoradiographic data:
  • Bilateral hilar adenopathy on the chest radiograph
  • Lofgren syndrome (erythema nodosum skin rash + bilateral hilar adenopathy on chest radiograph +/- fever and arthritis)

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