Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Y4 Respiratory > Restrictive lung disease > Flashcards

Restrictive lung disease Flashcards

1
Q

Define what restrictive lung disease is

A
  • This is where there is a decrease in the total volume of air that the lungs are able to hold.
  • Causes may be intrinsic (caused by disease of the lungs itself) or extrinsic (caused by disease outside of the lung - often related to disease affecting the expansion of the chest wall)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Describe the pathology & causes of intrinsic causes of restrictive lung disease

A

There is impaired alveolar gas exchange to O2 whilst CO2 gas exchange remains unimpaired. Due to inflammation &/or scar tissue, & aveolar inflammation caused by the interstitial lung diseases (ILD’s) or due to alveolar inflammation caused by oedema, haemorrhage (vasculitis) or infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Describe the pathophysiology & causes of extrinsic causes of restrictive lung disease

A

Causes:

  1. Thoracic/extra-thoracic = obesity, kypho-scoliosis, ascites, diaphrgmatic palsy
  2. Neuromuscular disorders = MND or myasthenia gravis
  3. Pleural diseases = diffuse pleural thickening, mesothelioma or large pleural effusions

These causes result in impaired alveolar ventilation (hypoventilation) which results in a rise in PaCO2 & a decrease in PaO2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How is the general diagnosis of restrictive lung disease made ?

A

A combination of clinical exammination, PFT’s & radiological assessment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How is the diagnosis of specific ILD’s made ?

A
  • This requires careful history taking regarding -Occupational, hobbies, smoking & drug history
  • Often then supported by blood tests & radiological CT patterns of disease, MDT’s & occasionally lung biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the PFT’s which show restrictive lung disease ?

A
  • Decreased FVC with a FEV1/FVC > 70
  • Decreased DLCO (<80% of predicted)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Recall what DLCO is

A

This measures the ability of the lungs to transfer gas from inhaled air to the red blood cells in pulmonary capillaries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

List the causes of a decreased DLCO

A
  • Anaemia
  • Emphysema
  • ILD’s
  • Pulmonary oedema
  • PE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is DLCO used to monitor ?

A

Treatment response in ILD’s (it is more senstive than FVC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the different Tx’s for the causes of extrinsic causes of ILD?

A
  • Lifestyle, weight loss Mx for obesity
  • Treatment of neuromuscular disease e.g. myridostigamine in MG
  • Intercostal or ascitic drainge for ascites or pleural effusion
  • Corrective spinal surgery for scoliosis
  • Decortication for chronic empyema
  • Diaphragmatic plication for diaphragmatic paralysis etc
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Define what the lung parenchyma is

A

This is the portion of lung involved in gas transfer i.e. the alveoli, alveolar ducts & resp bronchioles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Define what interstitial lung disease is

A
  • This is the generic term used to describe a number of conditions that primarily affect the lung parenchyma in a diffuse manner,
  • They are characterised by chronic inflammation &/or progressive interstitial fibrosis & share a number of clinical pathological features
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the 3 different classifications of ILD’s ?

A
  1. Those with a known cause
  2. Those associated with systemic disorders
  3. Idiopathic pulmonary fibrosis (unknown cause)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

List the known causes of ILD’s

A
  • Occupational/environmental e.g. asbestosis, beryllosis, silicosis, cotton workers lung (byssinosis), coal workers pneumoconiosis
  • Drugs e.g. nitrofuratonin, bleomycin, amiodarone, sulfasalazine & busulfan
  • Hypersensitivity reactions e.g. EAA
  • Infections e.g. TB, fungi, viral
  • GORD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

List the systemic disorders associated with causing ILD

A
  • Sarcoidosis
  • RA
  • SLE, systemic sclerosis, mixed connective tissue disease & sjorgens syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the signs/symptoms of ILD’s ?

A

Symptoms:

  • Progressive SOB
  • Dry cough
  • Malaise
  • Weight loss
  • Arthralgia

Signs:

  • Bilateral fine-end inspiratory crackles
  • Finger clubbing
  • Restrictive spirometry pattern & decreased DLCO
  • Abnormal CXR or CT
  • Cyanosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How should everyone with suspected ILD be investigated/ diagnosed ?

A
  • Detailed history + clinical exammination
  • Blood tests to help identify underlying cause + ABG
  • Spirometry & DLCO
  • CXR
  • HR-CT

MDT then review this info to made diagnosis or deicde on further tests

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What blood tests are done with investigating someone for ILD?

A
  • FBC, ESR, CRP
  • Immunoglobulins (connective tissue & vasculitis screen - ANCA’s with MPO/PR3 Abs)
  • ANA
  • Rheumatoid factor
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

If a confident diagnosis cannot be made by the MDT based on the initial tests then what is done to definitivley diagnose ILD?

A

Bronchoalveolar lavage or transbronchial biopsy &/or surgical lung biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Describe what extrinsic allergica alveolitis (EAA) is

A
  • It is a type of ILD, EAA is also known as hypersensitivity pneumonitis & is a condition where in sensitised individuals, inhalation of allergens (fungal spores or avian proteins) provoke a hypersensitivity reaction
  • In the acute phase (type III hypersensitvity) alveoli are inflated with acute inflam cells. With chronic exposure (type IV) hypersensitivity granuloma formation & oblierative bronchiolitis occur
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

List the causes of EAA

A
  • Bird-fanciers lung - avain proteins
  • Farmers lung - spores of saccharopolyspora
  • Mushroom workers lung - thermophilic actinomycetes
  • Malt workers lung - aspergillus clavatus
  • Bagassosis or sugar workers lung - thermoactinomyces sacchari
  • Drugs - gold, bleomcyin, sulfasalazine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are the acute features of EAA & when do they occur ?

A

4-8hrs after exposure to allergens:

  • SOB
  • Dry cough
  • Fever
  • Myalgia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the features of chronic EAA?

A

Features of ILD outlined previously

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are the Ix findings of ILD suggestive of EAA?

A
  • CXR - upper zone fibrosis (honeycomb lung)
  • Bronchoalveolar lavage - ly,phocytosis & mast cells
  • Bloods - neutrophilia, positive serum precipitins
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is the management of EAA?

A

Acute Mx = Remove allergen & give O2 then:

  • Give oral prednisolone
  • In cases of progressive fibrosis give anti-fibrotic therapy (Pirfenidone or Nintedanib)

Chronic:

  • Avoid future exposure to allergens or wear facemask or +ve pressure helment
  • Long-term steroids if breathless or low DLCO
  • May ve entitled to compensation (UK industrial injuries act)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Define what sarcoidosis is

A
  • It is a multi-system disorder of unknown aetiology characterised by non-caseating granuloma formation at various sites in the body with a predliction for the lungs & thoracic cavity.
  • It is possibly due to an imbalance of the immune system with type 4 hypersensitivity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Who is most commonly affected by sarcoidosis ?

A

Young adults & people of african decent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What are the common systems affects by sarcoidosis ?

A
  • Lungs
  • Lymph nodes
  • Joints
  • Liver
  • Skin
  • eyes
29
Q

Describe the typical progression of the disease if someone develops sarcoidosis

A
  • Upto 50% are asymptomatic & are diagnosed on the basis of routine CXR
  • Those who are symptomatic develop acute disease which can then go into remission but in roughly 10-30% they will develop a chronic, progressive pattern of disease
30
Q

What are the acute clinical features of sarcoidosis ?

A
  • Eryhthema nodosum
  • Bilateral hilar lymphadenopathy
  • Polyarthralgia
  • Uveitis
  • Parotitis (inflam of parotid glands)
  • Fever (swinging)
31
Q

What clinical feature is shown in the pic ?

A

Erythema nodosum

32
Q

What clinical feature is shown in the pic ?

A

Uveitis

33
Q

What are the constitutional symptoms which may be present in someone with sarcoidosis ?

A
  • Fever & night sweats
  • Malaise
  • Fatigue
  • Weight loss
34
Q

What are the features of chronic sarcoidosis ?

A
  • Lung infiltrates - dry cough, dyspnoea, bilateral hilar lymphadenopathy
  • Skin infiltrates - erythema nodosum, lupus pernio (chronic raised purpulish lesions affecting cheeks & nose)
  • Peripheral lymphadenopathy
  • Eye - anterior uveitis
  • Hypercalcaemia
  • Other organs - hepatitis, heart disease (cardiomyopathies, arrhythmias), neurological (bells palsy, lymphocytic meningitis) splenomegaly, renal (stones)
35
Q

How is sarcoidosis diagnosed?

A

There is no one diagnostic test, but generally needs histological evidence of granulomatous inflammation, exclusion of alternative causes & evidence of systemic disease

  • Bloods done - hypercalcaemia & increased ESR & serum ACE
  • Negative mantoux test
  • CXR which shows BHL +/- interstitial infiltrates
  • CT scan which shows peripheral nodular infiltrates
  • Tissue biopsy done (e.g. transbronchial, skin, lymph nodes) which hows non-caesating granulomas
  • PFT’s show restrictive disease pattern
36
Q

How is sarcoidosis staged ?

A

On CXR:

  • Stage 0 = normal
  • Stage 1 = BHL
  • Stage 2 = BHL + interstitial infiltrates
  • Stage 3 = Diffuse interstitial infiltrates only
  • Stage 4 = Diffuse fibrosis
37
Q

What is the management of sarcoidosis ?

A

1st line = oral steroids (prednisolone) if they are symptomatic stage ≥ 2 or 3 OR if hypercalcaemia, eye, heart or neuro involvement then treat regardless of stage or symptoms

38
Q

Do patients with sarcoidosis who have asymptomatic stage 2 or 3 disease with only midly abnormal lung function require treatment ?

A

No

39
Q

What are the 2nd and 3rd line treatment options for sarcoidosis ?

A
  • 2nd line = immunosuppressants e.g. methrotrexate or asathioprine etc
  • 3rd line = anti-TNF
40
Q

Define what idiopathic pulmonary fibrosis (IPF) is

A

It is a ILD characterised by progressive fibrosis of the interstitium of the lungs. Whilst there are many causes of ILD, the term IPF is reserved for when no underlying cause exists

41
Q

Who is IPF typically seen in ?

A

Patients aged 50-70 & more so in men

42
Q

What are the clinical features of IPF?

A

That of ILD:

  • Progressive dyspnoea
  • Bilateral fine end-inspiratory crackles/crepitations
  • Dry cough
  • Finger clubbing
  • Cyanosis
43
Q

How is IPF diagnosed ?

A
  • As previous flashcard for ILD, do all those Ix’s & then diagnose IPF only on the basis of MDT consensus
  • Then 2nd line as per ILD is a biopsy if needed
44
Q

What is the investigation of choice for investigating someone suspected of having IPF?

A

HR-CT

45
Q

Describe the pathology of IPF

A
  • Hetergenous fibrosis in alveolar walla with fibroblastic foci & destruction of archiecture causing honeycombing
  • Inflammation is minimal
46
Q

What CXR features are suggestive of IPF?

A

Bilateral interstitial shadowing - ground-glass appearance later progressing to honeycombing

47
Q

What is the management of IPF?

A
  • Pulmonary rehabiliation + supportive tx (oxygen) +/- pirfenidone or nintedanib
  • If no contraindications & young will eventually require lung transplantation
48
Q

What are the criteria which must be met for pirfenidone or nintedanib use in IPF?

A
  1. Person has a FEV between 50-80% predicted
  2. Company provides discount for the drug
  3. Tx is stopped if there is evidence of disease progression (decline of ≥ 10% in FVC within any 12-month period)
49
Q

What is the prognosis of IPF?

A

Poor - llife expectancy is around 3-4 years

50
Q

Define what pneumoconiosis is

A

This refers to a sub-group of ILD’s caused by the inhalation & retention of dusts in the lungs

51
Q

What are the main types of pneuoconiosis

A
  1. Coal workers pneumoconiosis (CWP) + Progressive massive pneumoconiosis
  2. Silicosis
  3. Asbestosis
52
Q

What is the cause of CWP + PMP?

A

It is caused by long-term exposure to coal dust particles

53
Q

What are the clinical features of CWP?

A
  • Usually asymptomatic, but CXR shows many round opacties esp in the upper zone
  • Restrictive LFT’s (spirometry) seen
  • Commonly have co-exisiting chronic bronchitis
54
Q

What is the management of CWP + PWP ?

A
  • Avoid exposure to coal dust
  • Treat co-exisiting chronic bronchitis
  • May be eligable for compensation via the industrial injuries act
55
Q

Describe what PWP is

A

This is where there is progression of CWP resulting in progressive dysponea, fibrosis & eventually cor pulmonale

56
Q

How is pneumoconiosis investigated ?

A

As per ILD guidelines

57
Q

What is the cause of silicosis ?

A

Inhalation of silicon dioxide (silica) particles

58
Q

What are the characteristic CXR features of silicosis ?

A

Diffuse miliary or nodular pattern in upper & mid zones and ‘egg-shell’ calcification of the hilar lymph nodes

59
Q

What occupation are at risk of silicosis ?

A
  • Mining
  • Slate workers
  • Foundries
  • Potteries
60
Q

What is the management of silicosis ?

A

Avoid exposure & claim compensation under industrial injuries act

61
Q

What are the clinical features of silicosis ?

A

Primarily progressive dyspnoea

62
Q

What causes asbestosis ?

A

Inhalation of asbestos fibres

63
Q

What jobs increase risk of asbestose exposure ?

A

Building trades

64
Q

What are the clinical features of asbestosis ?

A

Similar to all the other ILD’s:

  • Progressive dysponea
  • Fine end-inspiratory crackles
  • Finger clubbing
65
Q

What is the CXR features of asbestosis ?

A
  • Pleural plaques
  • Eventually diffuse pleural thickening and lower lobe fibrosis
66
Q

What does asbestosis increase the risk of ?

A

Mesothelioma & bronchical adenocarcinoma

67
Q

What is the management of asbestosis ?

A
  • Symptomatic Tx
  • Often eligable for compensation via the UK industrial injuries act
68
Q

Essentially what is the treatment of all the pneumoconioses?

A

Avoidance & compensation

69
Q

What is caplans syndrome ?

A

The association between RA, pneumoconiosis & pulmonary rheumatoid nodes

Y4 Respiratory (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions