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Clinical Pathology > Lecture 25 - Benign Respiratory Pathology > Flashcards

Lecture 25 - Benign Respiratory Pathology Flashcards

1
Q

What is asthma?

A

chronic inflammatory disorder of the airways.
Paroxysmal bronchospasm
wheeze
cough
variable bronchoconstriction that is at least partially reversible

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2
Q

is asthma reversible or obstructive or restrictive and is it reversible?

A

Asthma is an obstructive airway condition and is largely and partially reversible.

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3
Q

what are the symptoms of asthma?

A

mucosal inflammation and oedema
hypertonic mucous glands (high osmotic pressure than other bodily fluid) and mucus plugs in bronchi
hyperinflated lungs

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4
Q

what is the clinicopathological classification of asthma

A

4 types -

atopic, non-atopic, aspirin-induced, allergic bronchopulmonary aspergillosis (ABPA)

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5
Q

what are the features of atopic asthma

A

Type 1 hypersensitivity reaction

Persistent or irreversible changes

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6
Q

What happens in a type 1 hypersensitivity reaction?

A

Degranulation of IgE bearing mast cells

  • histamine initiated bronchoconstriction and mucus production obstructing air flow
  • eosinophil chemotaxis
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7
Q

what can cause a type 1 hypersensitivity reaction

A

Allergen - dust, pollen, animal products
cold, exercise, respiratory infections
many different cell types and inflammatory mediators involved

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8
Q

Persistent or irreversible changes in asthma

A

bronchiolar wall smooth muscles hypertrophy
mucus gland hyperplasia
respiratory bronchiolitis leading to centrilobular emphysema

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9
Q

Describe the epidemiology of Atopic asthma

A
  • Occurs in children and young adults
  • It is common
  • 33.9% UK children 12-14 years with “wheeze” in 2002.
  • 1 in 10 UK children diagnosed with asthma
  • 590,000 teenagers
  • 9-15% onset asthma is occupational
  • The commonest occupational lung disease
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10
Q

Acute asthma histology

A

a mucus plugged small bronchus with eosinophils

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11
Q

What causes localised or diffuse obstruction of air flow in Obstructive pulmonary disease?

A

Tumour or foreign body
Distal alveolar collapse or over expansion
Distal retention pneumonitis (endogenous lipid pneumonia ) and bronchopneumonia
Distal bronchiectasis (bronchial dilatation)

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12
Q

What is bronchiectasis?

A

Permanent dilation of bronchi and bronchioles caused by destruction of the muscle and elastic tissue

  • Results from chronic necrotizing infection
  • Rare
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13
Q

What is the site of bronchiectasis?

A

bronchus/bronchioles

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14
Q

Cause of bronchiectasis?

A

infection

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15
Q

What are the signs and symptoms of bronchiectasis?

A

cough, fever, copious amounts of foul smelling sputum

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16
Q

what are the predisposing conditions of bronchiectasis?

A

infections (immunocompromisation)
- cystic fibrosis
-primary ciliary dyskinesia or Kartagener syndrome
Bronchial obstruction: tumour, foreign body
-Lupus, rheumatoid arthritis, inflammatory bowel disease, GVHD

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17
Q

treatment for bronchiectasis

A

may be localised - resectable

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18
Q

Complications of bronchiectasis

A

Pneumonia, septicaemia, metastatic infection, amyloid

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19
Q

What is COPD?

A

A combination of chronic bronchitis and emphysema

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20
Q

What is chronic bronchitis?

A

cough and sputum for 3 months in each of 2 consecutive years

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21
Q

Site of bronchitis

A

bronchus

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22
Q

Cause of bronchitis

A

chronic irritation

smoking and air pollution

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23
Q

At what age is chronic bronchitis

A

Middle aged and old

1 in 20 of over 65 year olds consult their GP per year.

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24
Q

What is the pathology of chronic bronchitis

A

Mucus gland hyperplasia and hypersecretion, secondary infection by low virulence bacteria, chronic inflammation
-chronic inflammation of small airways of the lung causes wall weakness and destruction thus centrilobular emphysema

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25
Q

What is emphysema

A

Emphysema is a type of COPD involving damage to the air sacs (alveoli) in the lungs. As a result, your body does not get the oxygen it needs. Emphysema makes it hard to catch your breath. You may also have a chronic cough and have trouble breathing during exercise.

26
Q

Classification of emphysema

A

Centrilobular (centacinar) - due to coal dust and smoking

Panlobular (panacinar) - more than 80% alpha1 antitrypsin deficient, (rare, autosomal dominant), severest in lower lobe bases

Paraseptal (distal acinar)-
Upper lobe subpleural bullae adjacent to fibrosis. Pneumothorax if rupture.

27
Q

Site of emphysema

A

acinar

28
Q

Symptoms of emphysema

A

Dyspnoea: progressive and worsening

29
Q

Difference between Predominant bronchitis and emphysema

A 
Bronchitis 
age - 40-45
Dyspnea - Mild, late in disease
Cough - early in disease, copious sputum
Infections- common 
Respiratory insufficiency - Repeated
Cor pulmonale - common 
Chest Xray - Prominent vessels, large heart 
Stereotype - Blue bloater

Emphysema

50-75
Dyspnoea - severe, early 
cough - late, scanty, sputum
Infections - rare 
Respiratory insufficiency - terminal 
cor pulmonale - rare, terminal
Chest x-ray - Small heart, hyperinflated lungs
Stereotype - pink puffer
30
Q

what was observed in coal miners since 1992?

A

Chronic bronchitis and emphysema

31
Q

what caused chronic bronchitis and emphysema in coal miners

A

over 20 years of underground work
compensation depends on degree of disability and smoking history
No CXR or history of dust exposure needed

32
Q

Coal miners compensation scheme

A

4000 occupational disease deaths per year
592000 claims since 1993
575,000 settled
£2.3 billion paid in compensation

33
Q

What is interstitial lung disease?

A

restrictive rather than obstructive
very heterogenous group
diseases of pulmonary connective tissue
-Main alveolar walls

34
Q

what is the nature of Interstitial lung disease?

A

diffuse and chronic

35
Q

What are the causes of interstitial lung disease?

A

Causes often unknown

36
Q

what occurs in interstitial lung disease?

A
  • increased tissue in alveolar-capillary wall
  • inflammation and fibrosis
  • limited morphological patterns that differ with site and with time in any individual but with many causes and clinical associations
  • decreased lung compliance
  • increased gas diffusion distance
37
Q

What occurs in Acute interstitial lung disease?

A

Diffuse alveolar damage

  • exudate and death of type 1 pneumocytes from hyaline membranes lining alveoli followed by type 2 pneymocyte hyperplasia
  • Histologically acute interstitial pneumonia
  • Adult respiratory distress syndrome (shock lung)
  • shock, trauma, infections, smoke, toxic gases, oxygen paraquat, narcotics, radiation , aspiration, DIC
38
Q

What occurs in Chronic interstitial lung diseases?

A

Dyspnoea increasing from months to years
Clubbing, fine crackles, dry cough
Interstitial fibrosis and chronic inflammation with varying radiological and histological patterns
- Common end-stage fibrosed “honeycomb lung”
-examples
-idiopathic pulmonary fibrosis
many pneumoconioses (dust diseases)
- sarcoidosis
-collagen vascular diseases - associated lung diseases

39
Q

What is idiopathic pulmonary fibrosis

A

crytogenic fibrosing alveolitis

40
Q

Epidemiology of Idiopathic pulmonary fibrosis

A
  • 5000 new cases per year in uk, middle aged and elderly

- 3 & 5 year mortality 43% and 57% (expected 12% and 19%)

41
Q

What areas are affected in Idiopathic Pulmonary fibrosis?

A

Sub-pleural, lower lobes affected first and most severely

42
Q

Describe the histology of pulmonary fibrosis

A

Usual interstitial pneumonia (UIP) - Interstitial chronic inflammation & variably mature fibrous tissue

  • Adjacent normal alveolar walls
  • Similar pattern of fibrosis in collagen vascular disease associated interstitial lung disease and in asbestosis.
43
Q

What is idiopathic Pulmonary fibrosis?

A

Bosselated (‘cobblestone”) pleural surface due to contraction of interstitial fibrous tissue accentuates lobular architecture

44
Q

What is sarcoidosis?

A

Non-caseating perilymphatic pulmonary granulomas, then fibrosis
Hilar nodes usually involved
-other organs may be affected,-skin, heart, brain
- Hypercalcaemia and elevated serum ACE
- Typically young adult females, aetiology unknown

45
Q

What is pneumoconioses?

A

The dust diseases

  • Originally defined as the non neoplastic lung diseases due to inhalation of mineral dusts in the workplace
  • Now also includes organic dusts, fumes and vapours
46
Q

What are the different kinds of inhaled dusts

A 
inert
fibrogenic
allergenic
oncogenic
-causes lung carcinoma & pleural mesothelioma
47
Q

How big does dust have to be to reach the alveoli

A

less than 3Mm

48
Q

What is coal workers’ pneumoconiosis?

A

Also called Anthracosis (milder, asymptomatic Pneumoconiosis)

  • Simple (macular) CWP
  • Nodular CWP
  • Progressive massive fibrosis
  • COPD (‘ chronic bronchitis & emphysema’) if more than 20 years of underground mining
49
Q

What is coal worker’s pneumoconiosis associated with?

A

Right ventricular hypertrophy

50
Q

What is silicosis caused by?

A

Silica - sand and stone dust

51
Q

What occurs in silicosis?

A
  • Kills phagocytosing macrophages
  • Fibrous & fibrous silicotic nodules, also in nodes
  • Possible reactivation of tuberculosis
52
Q

What can silicosis increase risk of developing?

A
  • Increased risk of lung carcinoma - lung carcinoma with silicosis is a UK “ prescribed occupational disease”
  • Mixed dust pneumoconiosis - silica with other dusts
53
Q

What is hypersensitivity pneumonitis?

A
  • also called extrinsic allergic alveolitis
  • Type 3 hypersensitivity reaction organic dusts
  • Farmers’ lung - actinomycetes in hay
  • pigeon fancier’s lung - pigeon antigens
  • peribronchiolar inflammation with poorly formed non-caseating granulomas extends alveolar walls
  • repeated episodes leads to interstitial fibrosis
54
Q

What is cystic fibrosis?

A
  • An inherited multiorgan disorder of epithelial cells affecting fluid secretion in exocrine glands and the epithelial lining of the respiratory, gastrointestinal and reproductive organs
  • Mostly affects caucasians
  • Incidence 0.4 per 1000 live births
55
Q

What kind of inheritance is cystic fibrosis?

A

autosomal recessive inheritance

56
Q

What occurs in cystic fibrosis?

A
  • Bronchioles distended with mucus
  • Hyperplasia mucus secreting glands
  • Multiple repeated infections
  • Severe chronic bronchitis and bronchiectasis
  • exocrine gland ducts plugged by mucus
    -Atrophy and fibrosis of gland
    -Impaired fat absorption, enzyme secretion, vitamin deficiencies (pancreatic insufficiency)
    Small bowel: mucus plugging - meconium ileus
    Liver: plugging of bile cannaliculi – cirrhosis
    Salivary glands: Similar to pancreas: atrophy and fibrosis
    95% of males are infertile
57
Q

What bowel condition is associated with cystic fibrosis?

A

Meconium ileus - bowel obstruction when meconium is thicker and stickier than normal so causes obstruction of the ileum

58
Q

What are the screening processes available for CF?

A

Part of newborn screening in UK
sweat test
genetic testing

59
Q

What is the median age of survival in the uk for CF

A

41

60
Q

What is the treatment available for CF?

A

Physiotherapy
Mucolytics
Heart/lung transplants

61
Q

ethics of new drug for CF

A

The cost of lumacaftor–ivacaftor is £8,000 per 112‑tablet pack (excluding VAT; company’s evidence submission). The cost of a 1‑year course of treatment is £104,000 (excluding VAT). Costs may vary in different settings because of negotiated procurement discounts

Clinical Pathology (16 decks)

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