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RESPIRATORY > RESTRICTIVE LUNG DISEASE > Flashcards

RESTRICTIVE LUNG DISEASE Flashcards

1
Q

What drugs can cause ILD?

A

Amiodarone
Bleomycin and busulphan
Nitrofurantoin
Methotrexate
Cyclophosphamide

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2
Q

What is interstitial lung disease?

A

A term used to describe a number of conditions that primarily affect the lung parenchyma and are characterised by chronic inflammation and or progressive interstitial fibrosis

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3
Q

Whats the epidemiology of interstitial lung disease?

A

50 per 100,000 in the UK
IPF is most common with 6,000 new cases per year

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4
Q

Whats the pathophysiology of interstitial lung disease?

A

Inflammation and fibrosis in the lung interstitium due to fibroblasts secreting excess extracellular matrix secondary to damage.
The lung interstitium becomes thicker, increasing the diffiusion distance and impairing gas exchange

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5
Q

What are the 2 types of restrictive lung diseases?

A

Interstitial lung disease
Extra-pulmonary lung disease - stuctures around lungs are damage which prevents chest expansion

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6
Q

What are examples of extrapulmonary causes of restrictive lung diseases?

A

Pectus excavatum or pectus carinatum
Obesity
Pleural effusion
Myasthenia gravis
Kyphoscoliosis
Diaphragmatic hernia
Ascites
Pleural thickening

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7
Q

What are the classifications of interstitial lung diseases?

A

Those with known cause:
- occupational/envrironmental
- drugs
- hypersensitivity reactions
- infections
- GORD

Those associated with systemic disorders
- sarcoidosis
- rheumatoid arthritis
- SLE, systemic sclerosis, mixed connective tissue disease, Sjogren syndrome
- ulcerative colitis, renal tubular acidosis, autoimmune thyroid disease

Idiopathic :
- idiopathic pulmonary fibrosis
- cryptogenic organising pneumonia
- non-specific interstitial pneumonitis
- desquamative interstitial pneumonia
- acute interstitial pneumonia
- respiratory bronchiolitis-associated ILD

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8
Q

How can GORD cause interstitial lung disease?

A

Chronic micro aspiration can lead to inflammation and damage to the lung tissue
GORD also shares common risk factors with ILD such as smoking which may also contribute to the development of both conditions

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9
Q

What are occupational/environmental causes of interstitial lung disease?

A

Silica dust
Asbestos
Coal dust
Beryllium
Radiation

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10
Q

What drugs can cause interstitial lung disease?

A

Nitrofurantoin
Amiodarone
Bleomycin
Sulfasalazine
Busulfan

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11
Q

What are examples of causes of hypersensitivity pneumonitis?

A

Bird-fanciers lung is a reaction to bird droppings
Farmers lung is a reaction to mouldy spores in hay
Mushroom workers’ lung is a reaction to specific mushroom antigens
Malt workers lung is a reaction to mould on barley
Air-conditioner or humidifier lung - exposure to mould and bacteria growing in AC and humidifying systems
Chemical-induced - paints, adhesives, foams (chemicals such as isocyanates)
Woodworkers lung - exposure to wood dust e.g. sawing wood

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12
Q

What infections can cause interstitial lung disease?

A

Viral - infleunza, adenovirus, CMV, HSV, COVID
Bacterial - mycoplasma pneumoniae, legionella pneumophila and TB
Fungal - aspergillosis, histoplasmosis, cocidiomycosis
Parasitic - schistosomiasis, toxoplasmosis

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13
Q

How does sarcoidosis cause interstitial lung disease?

A

It triggers inflammation and the formation of granulomas in lung tissue which causes damage to the lungs

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14
Q

How does rheumatoid arthritis cause interstitial lung disease?

A

It affects up to 10% of people with RA
Believed to be an abnormal immune response which triggers inflammation and fibrosis in the lung tissues

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15
Q

How do connective tissue disorders cause interstitial lung disease?

A

ILD affects up to 30% of people with connective tissue disorders
Abnormal immune response triggers inflammation and fibrosis in the lung

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16
Q

Whats the most common type of interstitial lung disease?

A

Idiopathic pulmonary fibrosis - 50% of all cases

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17
Q

What is hypersensitivity pneumonitis?

A

Aka extrinsic allergic alveolitis
An inappropriate immune response to an allergen = inflammatory response = fibrotic changes with an upper lobe predominance (type 3 hypersensitivity reaction)

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18
Q

Whats the pathophysiology of hypersensitivity pneumonitis?

A

3 stages:
1. Sensitisation phase - repeated exposure to a specific antigen leads to the development of an immune response with the production of IgG and activation of T cells
2. Acute inflammation phase - exposure to antigen triggers an acute immune response in the lungs characterised by the influx of immune cells into the alveolar space and interstitium
3. Chronic inflammation - if antigen exposure persists then non-caseating granulomas and fibrosis in lung tissue forms. This can cause irreversible lung damage

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19
Q

What are the clinical features of hypersensitivity pneumonitis?

A

Acute phase - 4-6 hours post exposure - fevers, rigors, myalgia, dry cough, dyspnoea, fine bibasal crackles
Chronic - finger clubbing, increasing dyspnoea, weight loss, decreased exercise tolerance, type 1 respiratory failure, cor pulmonale

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20
Q

How do you diagnose hypersensitivity pneumonitis?

A

Bloods - FBC shows neutrophilia, raised ESR, ABGs, serum antibodies
CXR - upper zone fibrosis and honeycomb lung
CT chest - nodules, ground glass appearance, extensive fibrosis
Lung function tests - restrictive defect
Bronchoalveolar lavage - raised lymphocytes and raised mast cells

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21
Q

How does idiopathic pulmonary fibrosis typically present?

A

70 year old with exertional dyspnoea and dry cough and fatigue
Bilateral fine end-inspiratory crackles on auscultation, clubbing and acryocyanosis

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22
Q

What are characteristic changes on high-resolution CT seen in idiopathic pulmonary fibrosis?

A

Usual interstitial pneumonia :
Honeycombing
Reticular opacities
Traction bronchiectasis
Emphysema
Loss of lung volume
(Usually seen in bases and peripheries)

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23
Q

Whats the prognosis of idiopathic pulmonary fibrosis?

A

50% 5 year survival rate
2-5 year survival rate from diagnsosi

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24
Q

What are some industrial dust diseases?

A

Coal workers pneumoconiosis
Silicosis
Asbestosis
Berylliosis
Byssinosis
Hypersensitive pneumonitis

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25
Q

Which 2 medications can be used to slow the progression of idiopathic pulmonary fibrosis?

A

Pirfenidone - antifibrotic and anti-inflammatory
Nintedanib - monoclonal antibody targeting tyrosine kinase

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26
Q

What is cryptogenic organising pneumonia?

A

Aka bronchiolitis obliterans organising pneumonia
A type of interstitial lung disease that involves a focal area of inflammation of the lung tissue
Can be idiopathic or triggered by infection, inflammatory disorders, medications, radiation, environmental toxins or allergens

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27
Q

Whats the most common cause of hypersensitivity pneumonia is?

A

Farmers lungs - main antigen is thermophilic actinomyces species or aspergillosis species from moldy hay
Must be differentiates from ODTS in farmers

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28
Q

What are the differences between organic dust toxic syndrome and hypersensitivity pneumonitis?

A

Both resolut from exposure to organic dusts and share common symptoms of SOB and fever
ODTS is caused by toxic effects of endotoxins in organic dusts and hypersensitivity pneumonitis is a type 3 hypersensitivity immune response to organic antigens
ODTS is acute whilst HP can be acute or chronic
ODTS is managed primarily supportively and HP may require corticosteroids

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29
Q

How does smoking affect the risk of hypersensitivity pneumonitis?

A

Decreases the risk - may be because nicotine is immunosuppressive

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30
Q

What are the differences in presentation between acute and chronic hypersensitivity pneumonitis?

A

Acute - fever, cough, dyspnoea and fatigue within 12 hours of exposure. Symptoms will resolve within 48 hours of removing exposure
Chronic - progressive cough, dyspnoea, fatigue and weight loss. Characterised by fibrosis. Symptoms wont resolve fully with removal of exposure

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31
Q

Why are skin tests not helpful for hypersensitivity pneumonitis?

A

As skin tests test for IgE but in hypersensitivity pneumonitis it is actually an IgG mediated response

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32
Q

What are risk factors for idiopathic pulmonary fibrosis?

A

Cause isn’t known but thought to be some genetic risk factors involving surfactant, gel forming mucin and telomerase
Being older
Male sex
Cigarette smoking and environmental exposures
GORD - microaspirations
Obstructive sleep apnoea
Air pollution
Herpes infection
FHx

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33
Q

Whats the pathophysiology of IPF?

A

Recurrent microinjuries to alveolar epithelial and basement membrane
Release of pro inflammatory cytokines and chemo kinase e.g. TNF alpha
Activation of resident fibrocytes and recruitment of circulating fibrocytes
Pro-fibrotic chemicals e.g. PDGF and TGF beta are secreted by alveolar cells which stimulates fibroblast activation and differentiation into myofibroblasts
Myofibroblasts stimulate collagen synthesis
Fibrotic foci form -> fibrosed lung

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34
Q

What are the features of idiopathic pulmonary fibrosis?

A

progressive exertional dyspnoea
bibasal fine end-inspiratory crepitations on auscultation
dry cough
clubbing

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35
Q

What investigations should you do for IPF?

A

Bloods - CRP, ANA, rheumatoid factor
ABG - hypoxia and hypercapnia likely
Spirometry- restrictive pattern with reduced transfer factor
CXR
High resolution CT - investigation of choice and required for diagnosis

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36
Q

What might you see on x-ray for IPF?

A

CXR - small, irregular peripheral opacities - ground glass appearance that later progresses to honeycombing

37
Q

How do you manage IPF?

A

Pulmonary rehabilitation
Supportive care
Pharmacological interventions - there is no conclusive evidence to support the use of any drugs to increase survival - Pirfenidone may be useful in selected patients
Supplement oxygen
lung transplant

38
Q

Whats the prognosis for IPF?

A

3-4 years from diagnosis

39
Q

How do you investigate for hypersensitivity pneumonitis?

A

imaging: upper/mid-zone fibrosis
bronchoalveolar lavage: lymphocytosis
serologic assays for specific IgG antibodies
blood: NO eosinophilia

40
Q

How do you manage hypersensitivity pneumonitis?

A

Avoid precipitating factors
Oral glucocorticoids

41
Q

When do you typically see radiation-induced pulmonary fibrosis?

A

Typically follows radiotherapy for breast or lung cancer and is usually seen 6-12 months after radiotherapy course

42
Q

What are complications of idiopathic pulmonary fibrosis?

A

Type 2 respiratory failure
Increased risk of lung cancer
Cor pulmonale
50% mortality in 5 years

43
Q

What is asbestosis?

A

Lower lobe interstitial lung fibrosis that manifests in patients 15-30 years following exposure to asbestos

44
Q

How much exposure to asbestos do you need to get asbestosis?

A

A considerable amount - over a few months at least
You need much less to cause development of mesothelioma although this is still very rare

45
Q

How does asbestosis present?

A

Dyspnoea
Cough
Clubbing
bilateral end-inspiratory crackles on auscultation
Cyanosis
Reduced chest expansion

46
Q

How do you investigate asbestosis?

A

Pulmonary function tests - restrictive with reduced gas transfer pattern
Chest X-ray
High resolution CT
Bronchoscopy and biopsy
Open lung biopsy

47
Q

What are chest X-ray findings caused by asbestosis?

A

Linear interstitial fibrosis
Pleural plaques
Pleural thickening
Atelactasis

48
Q

What are pleural plaques?

A

The most common form of asbestos-related lung disease
Generally occur 20-40 years after exposure
Benign and dont undergo malignant change

49
Q

Which form of asbestos is the most dangerous?

A

Crocidolite (blue)

50
Q

What is coal workers pneumoconiosis?

A

Aka black lung disease
Occupational lung disease caused by long term exposure to coal dust particles
15-20 years after initialy exposure to coal dust

51
Q

Whats the pathophysiology of pneumoconiosis?

A

Coal dust (2-5 μm in size) is inhaled and enters the lungs.
The dust reaches the terminal bronchioles and there it is engulfed by alveolar and interstitial macrophages.
The dust particles are then moved by the macrophages via the mucociliary elevator and removed from the body as mucus.
In coal miners who are exposed over many years, the system is overwhelmed and the macrophages begin to accumulate in the alveoli, which starts an immune response, causing damage to the lung tissue.

52
Q

What are the 2 ways which coal workers pneumoconiosis can present?

A

Simple pneumoconiosis
Progressive massive fibrosis

53
Q

What is simple pneumoconiosis?

A

The commonest type of pneumoconiosis
Often asymptomatic
Presence increases the risk of other lung disease
May lead to progressive massive fibrosis

54
Q

What is pneumoconiosis?

A

accumulation of dust in the lungs and the response of the bodily tissue to its presence

55
Q

What is progressive massive fibrosis?

A

A severe and progressive form of pneumoconiosis
Characterised by the development of large areas of fibrosis
Round fibrotic masses are most commonly in the upper lobes.
The exact pathogenesis is not known.
Patients are often symptomatic and have both breathlessness on exertion and cough, some may have black sputum.
Lung function testing shows a mixed obstructive/restrictive picture.

56
Q

What is silicosis?

A

a fibrosing lung disease caused by the inhalation of fine particles of crystalline silicon dioxide (silica)

57
Q

What is silicosis a risk factor for?

A

TB
Lung cancer
COPD
Autoimmune disease
Kidney disease

58
Q

Why can silicosis cause secondary tuberculosis?

A

Silicosis can impair the function of macrophages so they are less effective at controlling TB infection

59
Q

Which occupations are at risk of silicosis?

A

Mining
Slate works
Foundries
Potteries

60
Q

What are X-ray features of silicosis?

A

upper zone fibrosing lung disease
‘egg-shell’ calcification of the hilar lymph nodes

61
Q

What is sarcoidosis?

A

A non-caseating granulomatous inflammatory condition that affects lungs, liver, eyes, skin, heart, kidneys, CNS, PNS, bones
It’s of unknown aetiology

62
Q

Who is sarcoidosis most common in?

A

Young adults 20-40
People of African descent
Women

(Other spike of incidence is in 60s)

63
Q

Whats the most commonly affected organ by sarcoidosis?

A

Lungs (affects over 90% individuals)

64
Q

How can sarcoidosis affect the lungs?

A

Mediastinal lymphadenopathy
Pulmonary fibrosis
Pulmonary nodules

65
Q

How can sarcoidosis present?

A

Resp - cough, SOB, chest pain, wheeze
Skin - erythema nodosum, lupus pernio
Lymphadenopathy
Fatigue, weight loss, malaise, swinging fever
Joint pain and stiffness
Eyes - uveitis, conjunctivitis

66
Q

How can sarcoidosis affect the liver?

A

Liver nodules
Cirrhosis
Cholestasis

67
Q

How can sarcoidosis affect the eyes?

A

Uveitis
Conjunctivitis
Optic neuritis

68
Q

How can sarcoidosis affect the skin?

A

Erythema nodosum
Lupus pernio
Granulomas in scar tissue

69
Q

How can sarcoidosis affect the heart?

A

Bundle branch block
Heart block
Myocardial muscle involvement

70
Q

How can sarcoidosis affect the kidneys?

A

Kidney stones due to hypercalcaemia
Nephrocalcinosis
Interstitial nephritis

71
Q

How can sarcoidosis affect the CNS?

A

Nodules
Pituitary involvement e.g. diabetes insipidus
Encephalopathy

72
Q

What is Lofgren’s syndrome?

A

an acute form of sarcoidosis
characterised by bilateral hilar lymphadenopathy, erythema nodosum, fever and polyarthralgia.
It usually carries an excellent prognosis

73
Q

What is Mikulicz syndrome?

A

Form of sarcoidosis

there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma

Now outdated term and is included within the diagnosis of sjogrens syndrome

74
Q

What is Heerfordt’s syndrome?

A

Aka uveoparotid fever
Parotid enlargement, fever and uveitis
Secondary to sarcoidosis

75
Q

How do you investigate sarcoidosis?

A

Raised serum ACE
Hypercalcaemia
Raised serum soluble interleukin-2 receptor
Raised CRP
Raised immunoglobulins
U&Es for kidney involvement
Urine dipstick for urine ACR
LFTs for liver involvement
Ophthalmology for eye involvement
ECG and echo for heart involvement
Ultrasound abdo for liver and kidney involvement

CXR - Hilar lymphadenopathy
High resolution CT thorax
MRI for CNS involvement
PET scan for active inflammation

Biopsy - histology showing non-caseating granulomas with epithelioid cells is gold standard

76
Q

How do we manage sarcoidosis?

A

No treatment if no or mild symptoms as often resolves spontaneously

Oral steroids 6-24 months (give bisphosphonates alongside)
Second line - methotrexate or azathioprine
Lung transplant is rarely requires in severe pulmonary disease

77
Q

What are indications for steroid management of sarcoidosis?

A

patients with chest x-ray stage 2 or 3 disease who are symptomatic
hypercalcaemia
eye, heart or neuro involvement

78
Q

Whats the prognosis of sarcoidosis?

A

Spontaneously resolves within 6 months in 60% of cases
In a small number of pt it progresses with pulmonary fibrosis and pulmonary hypertension
Death only rarely occurs when it affects the heart causing arrhythmias or the CNS

79
Q

Why is diagnosis of cryptogenic organising pneumonia often delayed?

A

Due to its similarities to infective pneumonia - SOV, cough, fever, lethargy, CXR shows focal consolidation

80
Q

How is a definitive diagnosis of cryptogenic organising pneumonia made?

A

Lung biopsy

81
Q

How do you treat cryptogenic organising pneumonia?

A

Systemic corticosteroids

82
Q

How is interstitial lung disease different from COPD and asthma?

A

These diseases affect the airways whereas interstitial lung disease affects the lung tissue itself
Onset and progression of symptoms tends to be slower in interstitial lung disease

83
Q

How does sarcoidosis cause hypercalcaemia?

A

Occurs in up to 20% of cases
Granulomas can produce 1-alpha-hydroxylase which can convert 25-hydroxyvitamin D to its activate form = increased calcium absorption from the gut and increased bone resorption = hypercalcaemia
Granulomas can also produce cytokines that stimulate osteoclasts

84
Q

Why does sarcoidosis increase ACE?

A

The cells that make up granulomas are known to produce ACE
Reason is not entirely known

85
Q

What is TLCO?

A

Transfer factor for carbon monoxide (aka diffuse capacity of carbon monoxide) - a measure of the conductance of gas transfer from impaired gas to RBC
Used to evaluate the function of the alveoli

86
Q

What causes increased transfer factor?

A

Conditions where there is increased exposure of alveolar contents to blood e.g. pulmonary haemorrhage, polycythemia, left to right shunts

87
Q

What is the transfer coefficient?

A

Transfer factor / alveolar volume

88
Q

Why is transfer factor important?

A

if the patient has a disease that causes a decrease in lung surface area, or has had a lung removed, then there is a decrease in transfer factor but there is a normal KCO. However, in conditions such as fibrosing alveolitis or emphysema, where there is damage to the lung parenchyma there is a reduction in both transfer factor and transfer coefficient. On a similar note, if a reduction in lung volume is due to an inability to expand the thorax (e.g. weakness) then the TLCO is low but the KCO is normal or increased.

RESPIRATORY (22 decks)

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