Brainscape's Knowledge GenomeTM

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


See full index

103-theme 4 > interstitial lung diseases > Flashcards

interstitial lung diseases Flashcards

1
Q

what is interstitial shadowing? 3

A
  • reticular pattern
  • too many lung markings
  • may be local or may diffuse
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is the interstitium? 5

A
  • supporting structures of the lung
  • alveolar endothelium
  • capillary endothelium
  • basement membrane
  • connective tissue
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what can the interstitium be thickened by? 3

A
  • fluid
  • cells
  • fibrosis (aberrant wound healing leads to excessive deposition in the interstitium)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is the extracellular matrix?

functions? 3

A

3D fibre mesh filled with macromolecules (collagen, elastin)

  • tensile strength/elasticity
  • low resistance for effective gas exchange
  • tissue repair/modelling
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

name environmental exposure ILDs? 2

A
  • occupational lung disease

- hypersensitivity pneumonitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

name an idiopathic ILD?

A

-idiopathic pulmonary fibrosis (prototypical ILD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

name 2 systemic inflammatory disease?

A
  • connective tissue disease

- sarcoidosis

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

what is the presentation of idiopathic pulmonary fibrosis? 5

A
  • slowly and progressive exertional dyspnoea
  • non productive cough
  • dry inspiratory bibasal velcro crackles
  • +/- clubbing on fingers
  • abnormal pulmonary function test results- restriction and impaired gas exchange
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what is the HRCT pattern of idiopathic pulmonary fibrosis? 4

A
  • basal distribution
  • sub-pleural
  • traction bronchiectasis
  • honeycombing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

give a general summary of IPF? 9

A
  • unknown cause
  • causes progressive, irreversible fibrosis and is fatal
  • limited to the lungs
  • affects lower and peripheral lung
  • minimal inflammation–> no role for steroids
  • is a disease of older age
  • more common in men
  • more common in smokers
  • biopsy may be needed
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

describe IPF in the UK? 5

A

600 new cases each year

  • 1/100 deaths in the UK
  • median survival is 3 years from diagnosis
  • 20% still alive at 5 year
  • no reliable way to predict prognosis so patients live with massive uncertainty
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what is the management for IPF? 3

A
  • assess suitability for anti-fibrotic drugs: can only be prescribed at specialist centres, aim is to slow disease progression, often poorly tolerated–> weight loss, GI upset
  • assess suitability for lung transplant, <65, no significant co-morbidities
  • offer best supportive care to all
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is hypersensitivity pneumonitis? 6

A
  • diffuse inflammation of parenchyma in response to inhaled oxygen
  • involve upper lobes
  • bird droppings
  • farmers lung= thermophilic fungi
  • aspergillus= ubiquitous fungi
  • antigen is unknown in 50% of cases
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what is the presentation of hypersensitivity pneumonitis? 4

A

-acute= SOB, cough, fever, crackle within 4-6 hours of heavy exposure, often misdiagnosed as infection

most cases develop only after may years of continuous or intermittent inhalation of inciting agent

  • subacute= gradual onset of symptoms, weight loss is common
  • chronic= insidious onset, history of acute episodes may be absent, incomplete resolution with removal of antigen, may lead to irreversible fibrosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

how do we diagnose hypersensitivity pneumonitis ? 4

A
  • serum precipitins (circulating IgG antibody-antigen complexes) to specific antigen may be helpful
  • infinite number of possible antigens
  • may be positive in asymptomatic individuals
  • negative results do not exclude HP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

how do we manage hypersensitivity pneumonitis? 3

A
  • avoidance of enticing antigen
  • usually steroid responsive in early disease
  • may progress to irreversible fibrosis
17
Q

describe drug induced ILD? 4

A
  • medication history is very important
  • many drugs but the most common are nitrofurantoin, amiodarone and methotrexate
  • always consider if medication may be implicated-> association between initiation of drug and disease onset is important
  • ILD may develop months-years after starting the drug
18
Q

describe connective tissue disease related ILD? 7

A
  • Rheumatoid arthritis
  • Scleroderma
  • Sjogren’s
  • Polymyositis
  • Younger patients
  • Female preponderance
  • Detailed Hx: dry eyes/ mouth, joint pain/ swelling, rashes
19
Q

describe what you would see in the bloods of connective tissue disease related ILD?

A
  • antinuclear antibodies (ANA)

- rheumatoid factor (RF)

20
Q

how do we manage connective tissue disease related ILD? 2

A
  • liaise with rheumatology

- treat underlying disease (biologics, steroids, immunosuppression)

21
Q

how do we diagnose ILD (summary)? 4

A
  • clinical assessment–> is there an identifiable cause? CTD symptoms, drugs, exposures
  • bloods –> antinuclear antibodies, rheumatoid factors, angiotensin-converting enzyme, spirometry/lung function tests
  • CXR–> reticular shadowing
  • HRCT pattern of disease—> the cornerstone of diagnosis, only 60% are diagnostic
  • lung biopsy–>enhances diagnosis but risk often outweighs benefit, may differentiate IPF from other potentially reversible causes
22
Q

how do we treat ILD (summary)? 3

A
  • remove cause
  • immunosuppression
  • anti-fibrotics for IPF
23
Q

what is sarcoid? 7

A
  • Multisystem granulomatous disorder
  • Non necrotising granulomas
  • Cause is unknown
  • 3x more common in Afro-Caribbean’s, more sever disease
  • Some familial clusters
  • Disease of the young (30-60)
  • Unpredictable clinical course
24
Q

what is the histology of sarcoid? 7

A
  • Characterised by granulomatous inflammation
  • Unknown foreign antigen stimulates immune response including:
    CD4+ T cells
  • Alveolar macrophages
  • Multi-nucleate giant cells
  • Organised into granulomas
  • Granulomas occur in TB and fungal infections, but in sarcoidosis they are non-necrotising
  • A biopsy demonstrating granulomas along with clinical picture is needed to confidently diagnose sarcoidosis
25
Q

describe lofgren’s syndrome? 5

A
  • Erythema nodosum
  • Bilateral lymphadenopathy
  • Arthralgia
  • Excellent prognosis
  • Usually, self-limiting
26
Q

describe pulmonary sarcoidosis? 5

A
  • May be symptomatic
  • Cough
  • Dyspnoea with exertion
  • Chest tightness
  • Systemic symptoms: fatigue, sweats, weight loss, fever
27
Q

how do we diagnose pulmonary sarcoidosis? 9

A
  • No single diagnostic test
  • Combination of clinical picture, exclusion of alternative diagnoses and ideally biopsy of affected tissue
  • Baseline tests: renal function, liver function, calcium, serum ACE, CXR, ECG
  • Serum angiotensin-converting enzyme (ACE):
  • Secreted by activated alveolar macrophages in granulomas
  • Low sensitivity, poor specificity
  • Polymorphisms in ACE gene variation in peripheral blood ACE levels
  • No correlation with CXR stage of disease
  • Biopsy everything! Skin, lymph nodes, blind endobronchial biopsies
28
Q

how do we treat pulmonary sarcoidosis with major organ involvement? 3

A
  • ocular disease not responding to topical treatment
  • cardiac
  • neurological
29
Q

how do we treat pulmonary sarcoidosis with pulmonary disease? 5

A
  • Often less severe than extra-thoracic disease with spontaneous remission common
  • Short-term symptomatic benefit
  • Long term effect on natural history of disease is not known
  • Corticosteroids for 6-24 months are mainstay:
    inhaled corticosteroids may provide symptomatic benefit
  • Additional immunosuppressants may be necessary
30
Q

explain obstructive spirometry? 4

A
  • Indicates the problem is in the airways
  • Useful diagnostically:
  • Reversible airflow of obstruction asthma
  • Fixed airflow obstruction in a smoker COPD
    only a few other diseases of the airways
31
Q

explain restrictive spirometry? 7

A
  • Indicates there is a problem, but is not useful diagnostically
  • Useful for monitoring change (acutely, chronically, indicates risk of ventilatory failure)
  • Severity indicator for ILD
  • Treatment criteria for IPF anti fibrotic only if FVC 50-80% predicted
  • Restriction of lung expansion or loss of lung volume
  • Directly measured by TLC requires a lung function lab
  • FVC reflects the TLC? Can be done in clinic and is useful for disease monitoring
32
Q

explain lung function tests in ILD? 6

A
  • Fibrotic lungs are small and stiff
  • Higher elastic recoil than expected for volume
  • FEV1/FVC ratio may be high, but may be normal
  • Decreased TLCO (gas transfer)
  • Impaired gas exchange due to disruption of alveolar- capillary interface
  • Reduced total surface area for gas exchange

103-theme 4 (19 decks)

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