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Respiratory > Clinical features and management of restrictive lung disease > Flashcards

Clinical features and management of restrictive lung disease Flashcards

1
Q

What is the physiological definition of a restrictive lung disease?

A

Forced vital capacity <80% of the predicted normal

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2
Q

How does a Restrictive lung disease affect the lungs generally

A

They restrict lung expansion

Leading to decreased lung volume and increased work of breathing

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3
Q

What tissues/organs generally cause a restrictive lung disease?

A

Lungs

Pleura

Nerves/muscles

Bones (skeletal)

(There are others)

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4
Q

What are the 3 big restrictive lung diseases?

A

Idiopathic pulmonary fibrosis

Sarcoidosis

hypersensitivity pneumonitis

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5
Q

What is an alternative term for these restrictive lung diseases?

A

Interstitial lung diseases

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6
Q

What pleural conditions are restrictive?

A

Pleural effusions

Pneumothorax

Pleural thickening

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7
Q

What skeletal conditions are restrictive?

A

Kyphoscoliosis

Ankylosing spondylitis

Thoracoplasty

Rib fractures

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8
Q

What neuromuscular conditions are restrictive?

A

Amyotrophic lateral sclerosis

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9
Q

What sub-diaphragmatic causes are there for restriction of the lungs?

A

Obesity

Pregnancy

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10
Q

Histologically, what kind of always happens in a restrictive lung disease?

A

Thickening of the interstitium

ie thickening of the alveolar walls, and spaces between alveolar wall and capillaries and all that stuff

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11
Q

There are over 200 interstitial lung diseases

What can they all lead to, if not treated?

A

Pulmonary fibrosis

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12
Q

What is pulmonary fibrosis?

A

Scarring and thickening of the lung tissue

permanent thickening of the interstitium

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13
Q

What is sarcoidosis?

A

Multisystem granulomatous disease of unknown cause

Characterised by non caseating granulomas (collections of inflammatory cells) in the lungs, skin or lymph nodes

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14
Q

Who is prone to sarcoidosis?

A

Adults > 40

Women > men

World wide

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15
Q

How do you initially investigate a patient presenting with possible sarcoidosis

A

History
Examination
CXR

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16
Q

A patient’s CXR strongly indicates sarcoidosis

How do you further investigate it?

A

Pulmonary function tests

Bloods
Urinalysis 
ECG 
TB skin test 
Eye exam

Bronchoscopy
Biopsy

17
Q

Describe the bronchoscopy process for investigating sarcoidosis

A

Bronchoscopy including transbronchial biopsies and endobronchial ultrasound

18
Q

What surgical biopsies can be used for sarcoidosis if needed?

A

Mediastinoscopy

Video assisted thoracoscopic lung biopsy (VATS)

19
Q

What general type of drugs is used to treat sarcoidosis?

A

Steroids

20
Q

When would sarcoidosis NOT be treated?

A

Mild disease, no vital organ involvement, normal lung function, few symptoms

21
Q

When would NSAIDs be used for treatment of sarcoidosis?

A

-Moderate level of disease-

Erythema nodosum / arthralgia

22
Q

When would topical steroids be used for treatment?

A

-Serious level of disease-

Skin lesions / anterior uveitis / cough

23
Q

When would systemic steroids be used to treat sarcoidosis?

A

Cardiac, neurological, eye disease not responding to topical Rx, hypercalcaemia

24
Q

What is the prognosis for sarcoidosis?

A

Pretty decent

<1% caucasians die

10-20% sustain permanent pulmonary or extra-pulmonary complications

25
Q

What respiratory complications are mainly seen with chronic sarcoidosis?

A

Progressive respiratory failure

Bronchiectasis

Aspergilloma, haemoptysis, pneumothorax

26
Q

What is the typical presentation for idiopathic pulmonary fibrosis?

A

Chronic breathlessness and cough

Typically older (60-70), mainly men

Failed Rx for “LVF” or infection

Clubbed fingers

Crackles

27
Q

What is the general prognosis for idiopathic pulmonary fibrosis?

A

Median survival 3 years

28
Q

What are the pharmacological options for idiopathic pulmonary fibrosis?

A

OAF - oral anti-fibrotic
Pirfenidone
Nintedanib

(Not sure if the drugs above are OAFs or different)

29
Q

What surgical option can be used for IPF?

A

Lung transplant

30
Q

Aside from drugs or surgery

What care do patients with IPF receive?

A

Refer to ILD clinic (Interstitial lung disease)

Palliative care

Respiratory (32 decks)

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