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Year 3 > Week Three - Case Two > Flashcards

Week Three - Case Two Flashcards

1
Q

what is the typical presentations of ILD

A

Often slowly progressive
Occasionally may mimic an acute pneumonia
Dry cough
Progressive shortness of breath
May have clubbing
May have diffuse inspiratory crackles
Wheeze, haemoptysis and chest pain are rare
Investigations
Restrictive respiratory pattern of spirometry
Abnormal CXR

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1
Q

what are the two main types of ILD

A

pulmonary fibrosis and sarcoidosis

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2
Q

what is pulmonary fibrosis characterised by

A

scar tissue in the lungs

decreased compliance giving a restrictive pattern in pulmonary function tests (FEV1/FVC >80%)

end stage is honeycomb lung - cystic spaces develop in fibrotic lungs

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3
Q

how can pulmonary fibrosis be classified due to involvement

A

localised

bilateral

widespread

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4
Q

what are the four main clinical features in fibrotic lung conditions

A

dry cough
dyspnoea
digital clubbing
diffuse inspiratory crackles

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5
Q

what is idiopathic pulmonary fibrosis / cryptogenic fibrosing alveolitis

A

relatively rare, progressive, chronic pulmonary fibrosis of unknown aetiology.

occurs in patients 45-65 and involves the lower lobes

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6
Q

what are the main features of idiopathic pulmonary fibrosis / cryptogenic fibrosing alveolitis

A

breathlessness, dry cough, considerable weight loss and fatigue/malaise.

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7
Q

what are the blood investigations done for ILD - pulmonary fibrosis

A

FBC (raised ESR), Rh factor (+ve in 50% of patients), ANA (30% +ve)

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8
Q

what does a CXR show for ILD - pulmonary fibrosis

A

irregular, reticulo-nodular shadows, often in lower zones, sometimes called reticulonodular pattern

CXR is often normal

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9
Q

what will a CT show for ILD - pulmonary fibrosis

A

Usually a “high resolution CT” (HRCT)
Ground-glass opacification
“Honeycombing”
“Mosaicism”

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10
Q

What kind of pattern is shown on lung function tests - spirometry for ILD - pulmonary fibrosis

A

restrictive pattern

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11
Q

what will an ABG show for ILD - pulmonary fibrosis

A

hypoxaemia

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12
Q

what is corticosteroid treatment often combined with

A

often combined with azathioprine

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13
Q

what is the combined immunosuppressive treatment used for ILDs

A

Combined immunosuppressive therapy can also be used including azathioprine and cyclophosphamide in conjunction with steroids.

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14
Q

what is the prognosis for idiopathic pulmonary fibrosis

A

most patients die within 5 years

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15
Q

how is sarcoidosis treated

A

treatment only indicated if disease is progressive and / or has significant symptoms
Prednisolone – 0.5mg/Kg/day is usually the recommended treatment, for one month, and then weaning to the lowest effective dose, reviewed every 6-12 months

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16
Q

what is coal workers pneumoconiosis

A

related to total coal dust exposure. it results from inhalation of coal dust over 15-20 years

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17
Q

what are the two syndromes of Coal worker’s Pneumoconiosis

A

simone pneumoconiosis and progressive massive fibrosis

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18
Q

what is simple pneumoconiosis, what are the symptoms and how is it diagnosed

A

Simple Pneumoconiosis– Usually asymptomatic, but can have co-existent bronchitis. Diagnosis is made from CXR on finding small, round opacities in the upper zones. Avoidance of exposure to further dust can stop it from progressing. Patients are entitled to claim compensation via the Industrial Injuries Act.

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19
Q

what is Progressive massive fibrosis, what is seen soon chest X rays and what are the symptoms

A

Large, round fibrotic nodules >10mm seen on CXR, usually upper zones. Nodules can become infected by tuberculosis. The associated emphysema is severe. Cough, dyspnoea and production of black sputum may be present (sputum black due to ruptured, cavitating lesions, hence the name “the black lung”). This can progress despite cessation of exposure to dust, there is no specific treatment. Patients will eventually develop pulmonary hypertension and cor pulmonale.w

20
Q

what is Caplan’s syndrome

A

is the associated between CWP (coal workers pneumoconiosis) and rheumatoid arthritis

21
Q

what are the clinical features of asbestosis

A

fibrosis, dyspnea, dry cough, clubbing and inspiratory crackles

22
Q

what kind of cancer is asbestos linked strongly with

A

mesothelioma

23
Q

what is extrinsic allergic alveolitis

A

hypersensitivity pneumonitis and is a widespread inflammatory reaction.

24
Q

what does extrinsic allergic alveolitis result from

A

It results from repeated exposure to antigens to which the individual has already been sensitised.

Examples of these antigens include:
Mouldy hay (Farmer’s lung)
Bird Faeces (bird fancier/pigeon fancier’s lung)
Cotton fibres (bysinossis)
Sugar cane fibres (bagassosis)

25
Q

what are the clinical features of extrinsic allergic alveolitis

A

Clinical features are SOB, cough, fever which occur hours after antigen exposure. Chronically, features include: weight loss, progressive dyspnoea, type 1 resp. Failure and cor pulmonale.

26
Q

what is Goodpasture’s syndrome characterised by

A

glomerulonephritis and respiratory disease together.

27
Q

what is Goodpasture’s syndrome driven by

A

It is driven by a type 2 hypersensitivity reaction whereby IgG anti-basement membrane antibodies attach to the glomerulus to cause glomerulonephritis but can also react with the alveolar membrane to cause pulmonary haemorrhage.

The three features to remember are:
Haemoptysis
Haematuria
Anaemia

28
Q

what is the treatment of Goodpasture’s

A

corticosteroids, but in some cases plasmapheresis to remove autoantibodies has shown great success

29
Q

what are the differentials for breathlessness, unremarkable routine blood results and normal echocardiogram?

A
  • Lung cancer
  • Interstitial lung disease
  • Sarcoidosis
  • Asbestos-related lung disease
  • Occupational lung disease
  • Bronchiectasis
30
Q

what respiratory conditions are associated with nail clubbing

A

lung cancer,
suppurative lung diseases (cystic fibrosis, bronchiectasis) and ILD

31
Q

what can the early findings of ILD be on a CXR

A

mild basal interstitial changes seen as white lung markings in between the ribs.

this is a very early sign and may not be easily appreciated on chest x-ray imaging

32
Q

what further investigations should be done to confirm diagnosis of ILD

A

high resolution CT scan and spirometry

33
Q

ILD is restrictive, what would the spirometry results look like?

A

FVC and FEV1 are low, but proportional so, so that the FVC/FEV1 ratio remains normal.

lung volumes are also reduced, as is the transfer factor which measures the lung’s ability to soak up oxygen

34
Q

what kind of fibrosis is usually seen

A

it is usually sub pleural, basal and at the end of the lung

35
Q

what is a definitive diagnosis based on

A

CT - honeycombing (fibrosis)

36
Q

what kind of diagnosis is Idiopathic pulmonary fibrosis

A

diagnosis of exclusion

37
Q

what crepitations are a feature of fibrotic lung disease

A

Bi-basal, fine, end-inspiratory crepitations are a pathognomonic feature of fibrotic lung disease. They are sometimes described as ‘velcro-like’

38
Q

what types of ILD is smoking associated with

A

Desquamative Interstitial Pneumonia (DIP) and Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD) forms of ILD.

39
Q

what are the ILD conditions with unknown causes

A

idiopathic pulmonary fibrosis

sarcoidosis

cryptogenic organising pneumonia

40
Q

what medications are known to cause ILD

A

methotrexate

Amiodarone

bleomycin

nitrofurantoin

41
Q

what is methotrexate

A

an anti-inflammatory known to cause ILD in the form of hypersensitivity pneumonitis

42
Q

what is Amiodarone

A

an anti-arrhythmic medication known to cause ILD leading to lung fibrosis

43
Q

what is bleomycin

A

a chemotherapy agent

44
Q

what is nitrofurantoin

A

an antibiotic known to cause ILD leading to lung fibrosis with lung term use

45
Q

what are there treatments of idiopathic pulmonary fibrosis

A

antifibrotics - pirfenidone and nintedanib (slow the progression by about 50%)

pulmonary rehabilitation

oxygen assessment

46
Q

what are the side effects of antifibrotics - pirfenidone and nintedanib

A

nausea, reflux, loss of appetite

pirfenidone can cause rashes

47
Q

what is the immunosuppression treatment for non specific interstitial pneumonia pattern

A

mycophenolate

prednisolone

48
Q
A

Year 3 (63 decks)

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