Benign Respiratory Pathology Flashcards

1
Q

What is asthma?

What features is it characterised by?

A

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

What is meant by paroxysmal bronchospasm?

A

bronchospasm is a sudden constriction of the muscles in the walls of the bronchioles

it is caused by substances released from mast cells or basophils when they degranulate under the influence of anaphylatoxins

paroxysmal means that it is a severe attack or sudden increase in intensity, usually recurring periodically

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3
Q

How does asthma tend to affect the lungs and bronchi?

A
  • mucosal inflammation & oedema
  • hypertrophic mucous glands & mucous plugs in bronchi
  • hyperinflated lungs
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4
Q

What are the different types of asthma?

A
  • atopic
  • non-atopic
  • aspirin-induced
  • allergic bronchopulmonary aspergillosis (ABPA)
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5
Q

What is the pathology of atopic asthma?

What tends to trigger it and what does this lead to?

A

type I hypersensitivity reaction

  • allergens - dust, pollen, animal products
  • cold, exercise, respiratory infections
  • there are many diferent cell types and inflammatory mediators involved
  • it involves degranulation of IgE bearing mast cells
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6
Q

What is the result of degranulation of IgE bearing mast cells in atopic asthma?

A

histamine initiated brochoconstriction & mucus production obstructing air flow

eosinophil chemotaxis

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7
Q

What type of changes occur in atopic asthma?

A

persistent or irreversible changes

  • bronchiolar wall smooth muscle hypertrophy
  • mucus gland hyperplasia
  • respiratory bronchiolitis leading to centrilobular emphysema
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8
Q

Who is most commonly affected by atopic asthma?

How many children have asthma and what tends to cause asthma in adults?

A

it affects children and young adults

1 in 10 UK children diagnosed with asthma

9 - 15% of adult-onset asthma is occupational

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9
Q

What would acute asthma look like at autopsy?

A

a mucus plugged small bronchus with eosinophils

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10
Q

What is meant by obstructive pulmonary disease?

A

localised or diffuse obstruction of air flow

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11
Q

What are examples of causes of localised obstructive pulmonary disease?

A
  • tumour or foreign body
  • distal alveolar collapse (total) or over expansion (valvular obstruction)
  • distal retention pneumonitis (endogenous lipid pneumonia) and bronchopneumonia
  • distal bronchiectasis (bronchial dilatation)
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12
Q

What is bronchiectasis?

What does it result from?

A

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

results from chronic necrotising infection

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13
Q

What is the site, cause and signs/symptoms of bronchiectasis?

A

site:

  • bronchus / bronchioles

cause:

  • infections

signs / symptoms:

  • cough
  • fever
  • copious amounts of foul smelling sputum
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14
Q

What are the predisposing conditions for infections causing bronchiectasis?

A
  • cystic fibrosis
  • primary ciliary dyskinesia , Kartagener syndrome
  • bronchial obstruction - tumour or foreign body
  • lupus, rheumatoid arthritis, inflammatory bowel disease, GVHD
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15
Q

What are the complications of bronchiectasis?

A
  • pneumonia
  • septicaemia
  • metastatic infection
  • amyloid
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16
Q

What is chronic obstructive pulmonary disease a combination of?

A

a combination of chronic bronchitis and emphysema

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17
Q

What is meant by chronic bronchitis?

What age does it tend to affect?

A

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

tends to affect middle aged and older people

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18
Q

What is the site and cause of chronic bronchitis?

A

site:

  • bronchus

cause:

  • chronic irritation
  • smoking and air pollution
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19
Q

what is the pathology of chronic bronchitis?

A
  • mucus gland hyperplasia and hypersecretion
  • secondary infection by low virulence bacteria leads to chronic inflammation
  • chronic inflammation of small airways of the lung causes wall weakness & destruction
  • this leads to centriobular emphysema
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20
Q

What are the 3 different types of emphysema?

Where are they found / what are their causes?

A

centrilobular:

  • caused by coal dust & smoking

panlobular:

  • >80% are a1 antitrypsin deficient
  • severest in lower lobe bases

paraseptal:

  • upper lobe subpleural bullae adjacent to fibrosis
  • pneumothorax if it ruptures
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21
Q

What is the site, cause and symptoms of emphysema?

A

site:

  • acinar

cause:

  • depends on the type
  • (coal dust and smoking)

symptoms:

  • dyspnoea - progressive and worsening
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22
Q

Complete the table for COPD

A
23
Q

What is interstitial lung disease?

What are the characteristics and who does it affect?

A

describes diseases of pulmonary connective tissue - mainly alvelolar walls

it is a restrictive (rather than obstructive) lung disease

it is usually diffuse and chronic and causes are often unknown

24
Q

How is tissue and morphology affected in interstitial lung disease?

A

there is increased tissue in the alveolar-capillary wall

there is inflammation and fibrosis

limited morphological patterns that differ with site and with time in any individual but with many causes & clinical associations

25
Q

How does interstitial lung disease affect the lungs and gas exchange?

A

there is decreased lung compliance

there is increased gas diffusion distance

26
Q

What is shown in this image?

A

interstitial lung disease

27
Q

What is shown in this image?

A

interstitial lung disease

28
Q

What is shown in this image?

A

interstitial lung disease

29
Q

What is meant by diffuse alveolar damage in acute interstitial lung disease?

A

exudate & death of type I pneumocytes form hyaline membranes lining alveoli followed by type II pneumocyte hyperplasia

this is histologically acute interstitial pneumonia

30
Q

What condition is diffuse alveolar damage in acute interstitial lung disease associated with?

What causes this?

A

adult respiratory distress syndrome (shock lung)

  • shock
  • trauma
  • infections
  • smoke / toxic gases
  • oxygen
  • paraquat
  • narcotics
  • radiation
  • aspiration
  • DIC
31
Q

What are the clinical features of chronic interstitial lung diseases?

A
  • dyspnoea increasing for months to years
  • clubbing, fine crackles & dry cough
  • interstitial fibrosis and chronic inflammation with varying radiological and histological patterns
  • common end-staged fibrosed “honeycomb lung”
32
Q

What are examples of chronic interstitial lung diseases?

A
  • idiopathic pulmonary fibrosis
  • many pneumoconioses (dust diseases)
  • sarcoidosis
  • collage vascular diseases and associated lung diseases
33
Q

What is another term for idiopathic pulmonary fibrosis?

Which age groups tend to be affected?

Which lobes are affected?

A

cryptogenic fibrosing alveolitis

affects the middle aged and elderly

the sub-pleural and lower lobes are affected first and more severely

34
Q

What is the histology seen in idiopathic pulmonary fibrosis?

A

usual interstitial pneumonia

  • 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
35
Q

What does the pleural surface look like in idiopathic pulmonary fibrosis?

A

bosselated (“cobblestone”) pleural surface due to contraction of interstitial fibrous tissue which accentuates lobular architecture

36
Q

How does sarcoidosis affect the lungs?

A

non-caseating perilymphatic pulmonary granulomas, and then fibrosis

hilar nodes are usually involved

37
Q

What other organs are affected by sarcoidosis?

What are the systemic effects and who tends to be affected?

A

may affect the skin, heart and brain

causes hypercalcaemia and elevated serum ACE

typically affects young adult females and aetiology is unknown

38
Q

What is meant by pneumoconises?

A

“the dust diseases”

non-neoplastic lung diseases due to inhalation of mineral dusts, organic dusts, fumes and vapours

39
Q

what are the different kinds of inhaled dusts?

What size must they be to reach the alveoli?

A
  • inert
  • fibrogenic
  • allergenic
  • oncogenic
    • lung carcinoma & pleural mesothelioma
  • must be <3 um in order to reach the alveoli
40
Q

What is meant by coal workers’ pneumoconiosis and anthracosis?

A

pneumoconiosis:

  • a disease of the lungs due to inhalation of dust
  • characterised by inflammation, coughing and fibrosis

anthracosis:

  • milder type of pneumoconiosis caused by accumulation of carbon in the lungs
  • due to repeated exposure to air pollution or inhalation of smoke or dust particles
41
Q

What are the different types of Coal workers’ pneumoconiosis?

A
  • anthracosis
  • simple (macular) CWP
  • nodular CWP
  • progressive massive fibrosis
  • COPD (‘ chronic bronchitis & emphysema) if > 20 years underground mining
42
Q

What is silicosis caused by?

A

silica - this is sand and stone dust

it kills phagocytosing macrophages

leads to fibrosis and fibrous silicotic nodules, also in nodes

43
Q

What are the risks associated with silicosis?

A
  • possible reactivation of tuberculosis
  • increased risk of lung carcinoma
44
Q

what is mixed dust pneumoconiosis caused by?

A

inhalation of silica with other dusts

45
Q

What is an alternative name for hypersensitivity pneumonitis?

What is this?

A

extrinsic allergic alveolitis

it is a type III hypersensitivity reaction to organic dusts

there is peribronchiolar inflammation with poorly formed non-caseating granulomas

repeated episodes lead to interstitial fibrosis

46
Q

What are 2 more common names for hypersensitivity pneumonitis and why?

A

farmers’ lung:

  • due to actinomycetes in hay

pigeon fanciers’ lung:

  • due to pigeon antigens
47
Q

What is cystic fibrosis?

Which group of people are more commonly affected?

A
48
Q

How does cystic fibrosis affect the bronchioles?

A
  • bronchioles are distended with mucus
  • there is hyperplasia of mucus secreting glands
  • multiple repeated infections
  • leads to severe chronic bronchitis and bronchiectasis
49
Q

How does cystic fibrosis affect exocrine glands?

What are the consequences of this?

A
  • exocrine gland ducts are plugged by mucus
  • leads to atrophy and fibrosis of the gland
  • this leads to impaired fat absorption, enzyme secretion, vitamin deficiencies and pancreatic insufficiency
50
Q

How does cystic fibrosis affect the small bowel, liver and salivary glands?

How does it affect fertility?

A
  • mucus plugging in the small bowel leads to meconium ileus
  • plugging of bile cannaliculi in the liver leads to cirrhosis
  • atrophy and fibrosis of salivary glands
  • 95% of males are infertile
51
Q

How is cystic fibrosis tested for?

A

it is part of newborn screening in the UK

it involves sweat test and genetic testing

52
Q

What is the median survival for cystic fibrosis?

How is it treated?

A

median survival is 41 years in the UK

treatment involves:

  • physiotherapy
  • mucolytics
  • heart / lung transplants
53
Q
A