Interstitial Lung Disease Flashcards

1
Q

What is the new name for interstitial lung disease?

A

Diffuse Parenchymal Lung Disease

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2
Q

How are interstitial lung diseases classed?

A

Idiopathic if the cause is not known

Classified according to cause if known

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3
Q

What are some known causes of interstitial lung diseases?

A

Connective tissue diseases- SLE, RA, Systemic sclerosis

Occupational exposures- Asbestosis, Silicosis

Granulomatous- Sarcoidosis, Hypersensitivity pneumonitis

Treatment induces- e.g. Amiodarone, Methotrexate, Nitrofurantoin, Bleomycin

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4
Q

What are some of the idiopathic types of interstitial lung disease?

A

Idiopathic pulmonary fibrosis

Cryptogenic organizing pneumoni

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5
Q

What is main approach when investigated interstitial lung diseases?

A

Must rule out interstitial lung disease due to known causes as these are potentially treatable

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6
Q

How do interstitial lung diseases often present?

A

Shortness of breath
Worsens on exertion
Dry cough

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7
Q

What is heart on auscultation for interstitial lung diseases?

A

Fine crackes

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8
Q

What occupations would you consider for the different causes of ILD?

A

Asbestosis- Builder
Berylliosis- Aircraft engineer
Silicosis- Sand blaster
Hypersensitivity pneumonitis- Farmer, Bird keeper

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9
Q

What might be a cause of ILD in a rheumatoid arthritis patient?

A

The RA itself

Or the methotrexate being used to treat it

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10
Q

What might be seen on auscultation?

A

Clubbing
Fine crackles
RHF signs

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11
Q

What might you think of in a patient with bluish discolouration of the skin and ILD?

A

Amiodarone induced fibrosis

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12
Q

What are some features of sarcoidosis?

A
ILD
Bilateral hilar lymphadenopathy
Eryhthema nodosum
Hypercalcaemia
Uveitis/Conjunctivitis
Swelling of the parotids
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13
Q

What investigations should be done for ILD?

A
CXR- Reticular infiltrates
HRCT of thorax- shows characteristic honeycombing
Spirometry- Restrictive pattern
TLCO- Reduced transfer factor
Bronchoalveolar Lavage
Lung biopsy to confirm diagnosis

Other investigations will depend upon the cause:
Drug review, ANTI- CCP, ANA, Anti- SM, ESR, CRP, Anti Jo-1 (polymyositis/dermatomyositis). Pleural effusions may also be seen in connective tissue diseases.

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14
Q

What spirometry changes are seen in ILD?

A
Restrictive pattern:
FVC decreased
FEV1 normal
FEV1: FVC >0.7
TLC goes down
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15
Q

What test measures the barriers to diffusion in the lung?

A

TLCO

There is a reduced transfer factor in ILD

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16
Q

What are some of the causes of upper lobe fibrosis? (Use mnemonic)

A
Coal workers
Hypersensitivity
Histiocytosis
Allergic bronchopulmonary aspergilliosis
Anklyosing spondlyitis
Radiation
Tuberculosis 
Silicosis

CHHAARTS- Top of the charts = Upper lobe

17
Q

What are some of the cause of lower lobe fibrosis? Use mnemonic

A
Rhemuatoid arthritis
Asbesosis
Idiopthic Pulmonary Fibrosis
Scleroderma
Exogenous drugs
18
Q

What is elevated in sarcoidosis?

A

ACE
Calcium

This is because the granulomas secrete vitamin D.

Other features- Bilateral hilar lymphadenopathy, Erythema nodosum

19
Q

What infective disease causes exertional dyspnoea and dry cough?

A

PJP

Sample with induced sputum, bronchoalveolar lavage

20
Q

Why might a bronchoalveolar lavage be done in ILD?

A

Hypersensitivity pneumonitis and sarcoidosis both show raised lymphocytes on BAL.

21
Q

What causes extrinsic allergic alveolitis? Who is it seen in?

A

Exposure to allergens in the airways resulting in a hypersensitivity reaction. This is hypersensitivity pneumonitis.

It is seen in:
Bird keepers- Bird fanciers lung
Farm workers
Mushroom workers (fungal allergen)
Sugar workers
22
Q

What is the treatment for ILD?

A

If causative- manage appropriately, avoid exposure to allergens if hypersensitivity pneumonitis, stop amiodarone/methotrexate/chemotherapy agents, immunosuppression for connective tissue disease, avoid occupational expsoures

Oxygen therapy
Stop smoking
Antifibrotic agents being trialed
Steroids for severe hypersensitivity reactions
Lung Transplantation
23
Q

Do patients always require treatment for sarcoidosis?

A

Not if asymptomatic

If symptomatic steroids, azathioprine, cyclophosphamide

24
Q

What are two antifibrotic agents being used for idiopathic pulmonary fibrosis?

A

Nintedanib- Tyrosine kinase inhibitor

Pirfenidone- Regulates TGF Beta.

25
Q

What are two complications of asbestos exposure?

A

Asbestosis

Mesothelioma

26
Q

What type of granulomas are seen in sarcoidosis

A

Non-caseating

27
Q

How is acute sarcoidosis treated?

A

Most resolve

Bed rest and NSAIDs

If don’t resolve steroids and then steroid sparing agents such as cyclosporine and azathioprine