Respiratory - Interstitial Lung Disease Flashcards

1
Q

What is interstitial lung disease?

A

A group of conditions that affect the lung parenchyma, causing inflammation and fibrosis.

Conditions that are classified as interstitial lung disease include:
- sarcoidosis
- hypersensitivity pneumonitis
- usual interstitial pneumonia

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2
Q

How is interstitial lung disease diagnosed?

A

High resolution CT of the thorax, showing ground glass appearance.

When diagnosis is unclear, lung biopsy can be used to confirm the diagnosis via histology.

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3
Q

What pattern of lung disease will ILD show on lung function tests?

A

Restrictive lung disease (FEV1/FVC >0.8)

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4
Q

How is ILD managed?

A

Poor prognosis and limited management as the damage is irreversible - treatment is supportive:
- home oxygen if hypoxic at rest
- smoking cessation
- physiotherapy and pulmonary rehabilitation
- pneumococcal and flu vaccine
- advances care / palliative care planning
- lung transplant

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5
Q

What is idiopathic pulmonary fibrosis?

A

Progressive pulmonary fibrosis with no clear cause, presenting with insidious onset of SOB and dry cough.

Examination can show bibasal fine inspiratory crackles and finger clubbing.

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6
Q

List some drugs that can induce pulmonary fibrosis.

A
  • amiodarone
  • cyclophosphamide
  • methotrexate
  • nitrofurantoin
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7
Q

Secondary to which other conditions can pulmonary fibrosis occur?

A
  • alpha-1 antitrypsin deficiency
  • rheumatoid arthritis
  • systemic lupus erythematosus (SLE)
  • systemic sclerosis
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8
Q

What is usual interstitial pneumonia (UIP)?

A

Commonest type of pulmonary fibrosis, usually of idiopathic aetiology.

Classic findings include:
- bibasal inspiratory crackles
- clubbing
- reduced chest expansion
- pulmonary hypertension

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9
Q

Outline the pathophysiology of hypersensitivity pneumonitis (AKA extrinsic allergic alveolitis).

A

Type 3 hypersensitivity reaction to environmental allergens causes parenchymal inflammation and destruction.

Examples of specific causes:
- bird-fanciers lung is a reaction to bird droppings
- farmers lung is a reaction to mouldy spores in hay
- mushroom worker’s lung is a reaction to specific mushroom antigens
- malt workers lung is a reaction to mould on barley

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10
Q

What is asbestosis?

A

Lung fibrosis related to the inhalation of asbestos, usually occurring decades after original exposure.

Asbestos is fibrogenic and oncogenic, so can cause several complications:
- lung fibrosis
- pleural thickening
- adenocarcinoma
- mesothelioma

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11
Q

What is sarcoidosis?

A

A granulomatous multi-system inflammatory condition, associated with chest symptoms and extra-pulmonary manifestations.

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12
Q

Presentation of sarcoidosis.

A

Two spikes in incidence - young adulthood and around age 60. Women and BAME more commonly affected.

  • cough
  • fever
  • fatigue
  • weight loss
  • erythema nodosum
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13
Q

What are the effects of sarcoidosis on the lungs (~90%)?

A
  • mediastinal lymphadenopathy
  • pulmonary fibrosis
  • pulmonary nodules
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14
Q

What are the effects of sarcoidosis on the liver (~20%)?

A
  • liver nodules
  • cirrhosis
  • cholestasis
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15
Q

What are the effects of sarcoidosis on the eyes (~20%)?

A
  • uveitis
  • conjunctivitis
  • optic neuritis
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16
Q

What are the effects of sarcoidosis on the skin (~15%)?

A
  • erythema nodosum
  • lupus pernio
17
Q

What are the effects of sarcoidosis on the heart (~5%)?

A
  • bundle branch block
  • heart block
  • myocardial muscle involvement
18
Q

What are the effects of sarcoidosis on the kidneys (~5%)?

A
  • kidney stones (hypercalcaemia)
  • nephrocalcinosis
  • interstitial nephritis
19
Q

What are the effects of sarcoidosis on the central nervous system (~5%)?

A
  • nodules
  • diabetes insipidus (pituitary involvement)
  • encephalopathy
20
Q

What are the effects of sarcoidosis on the peripheral nervous system (~5%)?

A
  • facial nerve palsy
21
Q

What are the effects of sarcoidosis on the bones (~2%)?

A
  • arthralgia
  • arthritis
  • myopathy
22
Q

What is Lofgren’s syndrome?

A

A specific presentation of sarcoidosis:
- erythema nodosum
- bilateral hilar lymphadenopathy
- polyarthralgia (pain in multiple joints)

23
Q

Give some differential diagnoses for sarcoidosis.

A
  • tuberculosis
  • lymphoma
  • hypersensitivity pneumonitis
  • HIV
24
Q

What blood test findings would be consistent with a diagnosis of sarcoidosis?

A
  • raised serum ACE
  • hypercalcaemia
  • raised CRP
25
Q

What imaging findings would be consistent with a diagnosis of sarcoidosis?

A

CXR - hilar lymphadenopathy

HRCT - hilar lymphadenopathy; pulmonary nodules

MRI - CNS involvement

PET scan shows active inflammation

26
Q

What is the GOLD STANDARD for sarcoidosis diagnosis?

A

Bronchoscopy with ultrasound guided biopsy of mediastinal lymph nodes.

Histology shows characteristic non-caseating granulomas, with epithelioid cells.

27
Q

How is sarcoidosis treated?

A

No treatment is patient has no or mild symptoms, as the condition will often resolve spontaneously.

Oral steroids are first line where treatment is required; patients should be given bisphosphonates to protect against osteoporosis while on long term steroids.

28
Q

What is the prognosis of sarcoidosis?

A

Spontaneously resolves within 6 months in approx. 60% of patients.

Can progress with pulmonary fibrosis and pulmonary hypertension.

Death in sarcoidosis usually occurs secondary to cardiac or CNS involvement.