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Clinical Pathology > Lecture 25 - Benign Respiratory Pathology > Flashcards

Lecture 25 - Benign Respiratory Pathology Flashcards

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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
What is emphysema
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
Classification of emphysema
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
Site of emphysema
acinar
28
Symptoms of emphysema
Dyspnoea: progressive and worsening
29
Difference between Predominant bronchitis and emphysema
``` 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
what was observed in coal miners since 1992?
Chronic bronchitis and emphysema
31
what caused chronic bronchitis and emphysema in coal miners
over 20 years of underground work compensation depends on degree of disability and smoking history No CXR or history of dust exposure needed
32
Coal miners compensation scheme
4000 occupational disease deaths per year 592000 claims since 1993 575,000 settled £2.3 billion paid in compensation
33
What is interstitial lung disease?
restrictive rather than obstructive very heterogenous group diseases of pulmonary connective tissue -Main alveolar walls
34
what is the nature of Interstitial lung disease?
diffuse and chronic
35
What are the causes of interstitial lung disease?
Causes often unknown
36
what occurs in interstitial lung disease?
- 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
What occurs in Acute interstitial lung disease?
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
What occurs in Chronic interstitial lung diseases?
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
What is idiopathic pulmonary fibrosis
crytogenic fibrosing alveolitis
40
Epidemiology of Idiopathic pulmonary fibrosis
- 5000 new cases per year in uk, middle aged and elderly | - 3 & 5 year mortality 43% and 57% (expected 12% and 19%)
41
What areas are affected in Idiopathic Pulmonary fibrosis?
Sub-pleural, lower lobes affected first and most severely
42
Describe the histology of pulmonary fibrosis
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
What is idiopathic Pulmonary fibrosis?
Bosselated ('cobblestone") pleural surface due to contraction of interstitial fibrous tissue accentuates lobular architecture
44
What is sarcoidosis?
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
What is pneumoconioses?
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
What are the different kinds of inhaled dusts
``` inert fibrogenic allergenic oncogenic -causes lung carcinoma & pleural mesothelioma ```
47
How big does dust have to be to reach the alveoli
less than 3Mm
48
What is coal workers' pneumoconiosis?
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
What is coal worker's pneumoconiosis associated with?
Right ventricular hypertrophy
50
What is silicosis caused by?
Silica - sand and stone dust
51
What occurs in silicosis?
- Kills phagocytosing macrophages - Fibrous & fibrous silicotic nodules, also in nodes - Possible reactivation of tuberculosis
52
What can silicosis increase risk of developing?
- Increased risk of lung carcinoma - lung carcinoma with silicosis is a UK " prescribed occupational disease" - Mixed dust pneumoconiosis - silica with other dusts
53
What is hypersensitivity pneumonitis?
- 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
What is cystic fibrosis?
- 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
What kind of inheritance is cystic fibrosis?
autosomal recessive inheritance
56
What occurs in cystic fibrosis?
- 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
What bowel condition is associated with cystic fibrosis?
Meconium ileus - bowel obstruction when meconium is thicker and stickier than normal so causes obstruction of the ileum
58
What are the screening processes available for CF?
Part of newborn screening in UK sweat test genetic testing
59
What is the median age of survival in the uk for CF
41
60
What is the treatment available for CF?
Physiotherapy Mucolytics Heart/lung transplants
61
ethics of new drug for CF
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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