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Block 3 - Resp > Lecture 14 - Pathophysiology Of Interstitial Lung Disease > Flashcards

Lecture 14 - Pathophysiology Of Interstitial Lung Disease Flashcards

1
Q

Restrictive lung disease

A
  • reduced lung volume
  • Pulmonary: interstitial lung disease
  • Extrapulmonary: pleural disease, chest wall abnormality, neuromuscular disease
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2
Q

Defining ILD

A
  • lung disease characterised by damage to the interstitium of the lung by barying degrees of inflammation and fibrosis
  • includes a diverse cluster of parenchymal lung diseases
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3
Q

Interstitium

A
  • space between epithelial and endothelial basement membranes
  • space betweel alveoli and the capillary bed
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4
Q

MEchanism of ILD

A
  • depends on the cause of the ILD
  • lung responds to damage by attempting to repair
  • if known source not treated or removed then lung healing is delayed or prolonged
  • if imperfect: permanent damage
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5
Q

ILD of known cause or association

A
  • Exposure: Occupation, envt, medication, drug, radiation, smoking
  • systemic disease: CTD, IBD, sarcoidosis
  • Genetic: Familial, HPS
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6
Q

ILD of unkown cause

A
  • IIP: IPF, NSIP, COP, LIP

- Specific pathology: LAM, PAP

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7
Q

Why does the exact diagnosis matter?

A
  • prognosis

- treatment

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8
Q

Prgnosis and ILD

A
  • IPF, fibrotic NSIP are bad
  • cellular NSIP, sarcoidosis or drug induced have a better prognogis
  • early diagnosis is important - no difference in survival of NSIP and IPF in patients with severe disease
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9
Q

Impact of appropriate diagnosis on management

A
  • IPF: antifibrotic drug, lung transplantation, clinical trials, supportive care
  • non IPF ILD : corticosteroids and or immunosuppressive therapy. Lung transplant not possible
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10
Q

5 key considerations in ILD

A
  • does the patient have an ILD?
  • is it idiopathic or are there systemic features?
  • Is it IPF?
  • how severe is the disease overall?
  • what is the rate of disease progression?
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11
Q

History for ILD

A
  • exposure: smoking, medication, occupation, hypersensitivity triffers
  • connective tissue symptoms (may be present in up to 20% of patients)
  • GORD, dysphagia
  • Sarcoid, vasculitic symptoms
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12
Q

Physical examination

A
  • 80% have fine crackles on inspiration
  • inspiratory squeaks in primary bronchiolitis, HP
  • Clubbing - more common in IPF, chronic HP, DIP, HCX, - rare in RB-ILD, CVD, sarcoid, COP or LIP
  • pulmnonary hypertension
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13
Q

Blood and Urine tests

A
  • FBC, UEC, LFT, ESR, CRP, Ca/PO4
  • CK, BNP, ACE
  • autoimmune: ANA, ENA, dsDNA, RF, ANCA, antiCCP, anticentromere, myositis screen
  • Immunoglobulins, TPMT
  • Hypersensitivity screen - precipitins or antibodies
  • specific serlogy
  • Urine: MSU
  • 24 hr Urine: protein/Ca
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14
Q

Pulmonary function test

A
  • not specific for diagnosis but useful to assess disease severity

RESTRICTIVE PICTURE
- TLC

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15
Q

DLco and KCO

A
  • DLco is the diffusing capacity. It is reduced in ILD
  • KCO = DLco/VA
  • KCO is relatiely preserves as both variables are decreased
  • KCO is reduced when there is concomitant emphysema or pulmonary hypertension
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16
Q

High Resolution CT chest

A
  • Helpful in diagnosis of ILD

- highly specialised field

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17
Q

Idiopathic pulmonary fibrosis pattern

A

= UIP pattern

  • patchy - areas of normal lung
  • thin walls at the base of the lung
  • pattern is basal + peripheral
18
Q
  • Non-specific intersticial pneumonitis
A
  • grey fuzziness

- quite homogenous

19
Q

Respiratory bronchiolitis interstitial lung disease

A
  • tiny nodules that are scattered
20
Q

Desqamative interstitial pneumonia

A
  • grey fuzziness
  • tiny little custs
  • severe
21
Q

Lymphocytic interstitial pneumonia

A
  • big thin wall cysts

- histology: plenty of infiltrated lymphocytes

22
Q

Bronchoscopy

A
  • airways usually look normal
  • BAL: lymphocytosis in sarcoid, hypersenstivity. Helps confirm diagnosis and exclude infection
  • Transbronchial lung biopsy: neoplastic cells or sarcoid, cryobiopsy
  • EBUS: combined nodaul aspirate and TBBx can increase the yield for sarcoidosis to 80% +
23
Q

Surgical lung biopsy

A
  • only 10-20% undergo biopsy
  • mortality in up to 2%
  • complications in 5-10%
  • diagnostic yield is 50-90%
24
Q

Surgical lung biopsy useful when

A
  • non-classic HRCT appearance
  • atypical presentation
  • clinically fit for surgery
  • always biopsy two distant sites
  • changes diagnosis in 50-70% of non-IPF cases, but rarely in classic IPF
25
Q

Other tests in ILD

A
  • 6 min walk test: distance + oxyen desaturation
26
Q

Three pillars of treating ILD

A
  • Disease specific therapy: pharmacological treatments, pulmonary rehab, management of comorbidities
  • symptom based management: dyspnoea, cough
  • Patient education: support groups, participation in care
27
Q

Disease centered management targets for IPF therapy

A
  • prevent AEC apoptosis
  • block TGF-b production/activation
  • prevent fibrocyte recruitment
  • inhibit fibroblast activation
  • Prevent EMT
  • prevent injury
  • block TGFb activity
  • prevent telomere shortening
28
Q

Pirfenidone

A
  • novel antifibrotic
  • inhibits TGF-b induced collagen synthesis
  • blocks the mitogenic effect of PDGF and TGF-b on lung fibroblasts
  • reduces lung fibrosis in animal models
  • reduces mortality and IPF related deaths
  • reduces any serios adverse event
  • increase adverse events leading to treatment discontinusation (GIT side effects and skin side effects)
29
Q

Nintedanib in IPF

A
  • intracellular tyrosine kinase inhibitor
  • PDGF, FGF, and VEGF receptor blocker
  • inhibits belomycin-induced lung fibrosis in rat
  • decreased rate of decline in FVC by 50%
  • decrease acute exacerbation
  • non-significant decrese in QOL
  • non-significant decrease in Mortality
  • moe adverse events than placebo, as well as more drug-related AE
  • more adverse events leading to discontinuation
  • no difference in severe AE and fatal AE
30
Q

Main adverse event with Nintadenadib

A
  • Diarrhoea
  • mostly mild or moderate intensity
  • premature discontinuation in
31
Q

Pulmonary rehabilitation

A
  • short term gain in 6Min Walking distance and Quality of life
  • more effective in early disease
32
Q

IPF is associated with a number of comorbidities

A
  • Pulmonary hypertension
  • cardiovascular disease
  • GORD reflux
  • Sleep disorders
  • psychiatric disorder (depression/anxiety) - almost universal
33
Q

Symptom management: Dyspnoea

A
  • Dyspnoea is so multifactorial. Can also lead to muscle weakness from deconditioning, depression and anxiety
  • management: regularly assess and educate, refer for pulmonary rehab early and often, evaluate for comorbidities
34
Q

Cough

A
  • common feature
  • often sign that leads to investigations and diagnosis
  • often more troubling feature
  • impact greatly on QOL
  • consider antitussives including codein
35
Q

Fourth pillar: transplantation

A
  • even for those in early stage of disease

- work up allows for listing once patient deteriorates

36
Q

Hypersensitivity pneumonitis`

A
  • immune reaction to inhaled antigens
  • two hit hypothesis
  • present with: sudden SOB, chest pain, fevers, chills, cough, malaise
  • in chronic HP patients may have symptoms that are very mild and do not present for months or years after exposure
37
Q

Hypersensitivity pneumonitis - treatment

A
  • need to remove exposure to particular antigen

- medications - corticosteroid treatment used to suppress immune response

38
Q

Idiopathic pulmonary fibrosis

A
  • specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause
  • most common idiopathic interstitial pneumonia
  • pulmonary manifestation only
  • specific radiologic and histopathological features
39
Q

IPF presentation

A
  • progressive breathlessness
  • mild cough, perhaps worsening at the time of diagnosis - trigger for investigation
  • decreased exercise tolerance
  • long asymptomatic period
  • more common in men
  • age of onset >50
  • survival 3-5 years from diagnosis
40
Q

Radiology and pathology of OPF

A
  • very specific radiologic picture
  • commonly agreed upon criteria by expert groups associated with ATS.ERS
  • most prominent radiological feature are honeycomb cysts
  • clinical-pathological-radiological concencus diagnosis
  • lung biopsy not required for diagnosis
41
Q

Pathogenesis of IPF

A
  • poorly understood
  • previously thought to be inflammatory process leading to fibrosis
  • may be suscpeitble individual
  • aberrant healing response
  • repetitive alveolar injury
  • laying down of collagen and the ECM

Block 3 - Resp (44 decks)

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