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Respiratory Flashcards

1
Q

What is Ivacaftor? What is it used for?

A

IvaCaFToR is used in cystic fibrosis and potentiates the CFTR, approved for certain mutations e.g. G551D.

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2
Q

What is the criteria for a primary pneumothorax (3)?

A
  1. Age < 50yrs
  2. Non-smoker
  3. No lung disease
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3
Q

For a pneumothorax, how much faster is the air reabsorbed by giving supplementary oxygen?

A

x4

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4
Q

What is the best evidence-based management of idiopathic pulmonary fibrosis?

A

Clinical trials.

Closely monitor PFT - transplant if TLCO = 40%

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5
Q

When should a patient with idiopathic pulmonary fibrosis be referred for lung transplantation?

Which is preferable single vs. double lung transplant?

What is the cut-off age?

A

When TLCO = 40%

Single lung transplants are preferable

Age cut-off is 65 yrs

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6
Q

Which of the following medications are useful in the treatment of idiopathic pulmonary fibrosis:

Steroids
Immunomodulators
Anti-fibrotics
NAC
Warfarin
A

None.

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7
Q

Give the DDx for the following non-specific findings often found in interstitial lung disease:

  1. Upper or mid-lung changes
  2. Peribronchovascular changes
  3. Extensive ground glass
  4. Micronodules
A
  1. Upper or mid-lung changes:
    sarcoidosis
    hypersensitive pneumonitis
  2. Peribronchovascular changes:
    sarcoidosis
  3. Extensive ground glass:
    NSIP
  4. Micronodules:
    hypersensitive pneumonitis
    MAC
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8
Q

What is another name for Constrictive Bronchiolitis?

A

Bronchiolitis Obliterans

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9
Q

What are the causes of Contrictive Bronciolitis (aka Bronchiolitis Obliterans)?

A

Many.

Cryptogenic

OR

Associated with Infection / connective tissue disease / inhalation trauma / drugs / allograft recipients….

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10
Q

Compare direct and indirect asthma bronchial challenge test.

Outline the component of airway hyperresponsiveness (AHR) that each tests.

Which of these test is specific vs. sensitive?

A

Indirect challenge:

  • mannitol, hypertonic saline, voluntary hyperventilation, exercise challenge.
  • Activate mast cells to release histamine/other mediators to bronchocontrict.
  • Specific tests for ‘inflammatory’ component of AHR.

Direct challenge:

  • methacholine, histamine
  • Directly constrict airway smooth muscle via receptors.
  • Sensitive test for ‘airway calibre’ component of AHR.
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11
Q

True/False: cryptogenic organising pneumonia (COP) is steroid responsive.

A

True.

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12
Q

What are the causes of Cyptogenic organising pneumonia (COP)?

A

Idiopathic

OR

Non-specific response to infection/drugs

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13
Q

What are the PFT findings of Cyptogenic organising pneumonia (COP)?

A

Mild-moderate restriction.

Reduced TLCO

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14
Q

Describe 4 findings on CT-chest in Cyptogenic organising pneumonia (COP).

A
  1. Consolidation of lower/subpleural zones
  2. Ground glass opacities
  3. Pulmonary nodules
  4. Pleural thickening
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15
Q

Semantically, is IPF (idiopathic pulmonary fibrosis) synonymous with UIP (usual interstitial pneumonia)?

A

No - though often used interchangeably.

IPF is the term for the clinical syndrome associated with the morphologic pattern of UIP

IPF is the most common of the 7 types of idiopathic interstitial pneumonia (IIP).

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16
Q

What is best measure of deterioration in IPF?

A

10% reduction in FVC on PFT

(previously: 15% reduction in DLCO)

Desaturation only occurs in advanced disease, therefore not useful.

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17
Q

Give 2 biomarkers of poor prognosis in IPF/UIP.

A
  1. Circulating fibrocytes suggests poor prognosis in IPF

2. Elevated serum CCL-18 (chemokine C-C motif ligand 18) suggests disease progression.

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18
Q

What is the diagnostic criteria for IPF/UIP (3)?

Which is more important HRCT findings or histopathology in the diagnosis of IPF?

A
  1. Exclude other causes of ILD
  2. UIP on HRCT (if not biopsied)
  3. Combination of HRCT and biopsy patterns

NB: HRCT trumps histopathology

  • Histopathology equivocal but HRCT is UIP then UIP.
  • HRCT with UIP then no need for biopsy.
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19
Q

What are the HRCT findings of IPF/UIP (8)?

A

PHaF-BRATS (8):

Patchy
Honeycombing
Fibrosis
Basal predominance
Reticular changes
Architectural distortion
Traction bronchiectasis
Symmetrical
20
Q

What is the histopathology of IPF/UIP (3)?

A
  1. Fibrosis and fibroblastic foci
  2. Patchy (areas of normal lung) and heterogenous
  3. Mostly collagen with minimal inflammatory cells.
21
Q

True/False: IPF/UIP histopathology is predominantly inflammatory cells.

A

False.

Mostly collagen, inflammatory cells are minimal.

22
Q

What are the 7 types of idiopathic interstitial pneumonia?

A

Mnemonic: ‘All Idiopathic Chronic Lung Disease aRe Nonspecific’

A: acute interstitial pneumonia (AIP).
The only acute process in the list.

I: idiopathic pulmonary fibrosis / usual interstitial pneumonia (IPF / UIP)

C: cryptogenic organizing pneumonia (COP)

L: lymphoid interstitial pneumonia (LIP)

D: desquamative interstitial pneumonia (DIP)

R: respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)

N: non-specific interstitial pneumonia (NSIP)

23
Q

True/False: in NSIP (non-specific interstitial pneumonia) there is ground glass changes and honeycombing on HRCT.

A

False.

Ground glass changes present but honeycombing is absent.

24
Q

True/False: IPF/UIP does NOT have ground glass changes on HRCT.

25
Q

Which 2 features differentiates UIP from NSIP on HRCT?

A

UIP: honeycombing but NO ground glass
NSIP: ground glass but NO honeycombing

Remember: hUney/UIP and grouNd/NSIP

26
Q

True/False: NSIP, similar to UIP, is also basal/subpleural sparing of the apices, symmetrical, with traction bronchiectasis and a reticular patient.

27
Q

What is the histopathology of NSIP (2)?

A
  1. Inflammatory cells with ABSENCE of fibroblastic foci.

2. Homogenous pattern and not patchy

28
Q

What is NSIP (non-specific interstitial pneumonia)?

A

Mainly connective tissue related lung fibrosis, may also be idiopathic.

Spectrum of fibrosis vs. ground glass (think inflammatory) changes.

29
Q

True/False: medical therapy is NOT effective in NSIP (non-specific interstitial pneumonia)?

A

False.

NSIP (unlike IPF/UIP) may be responsive to prednisolone/cyclophosphamise (if inflammation > fibrosis)

30
Q

Lymphoid interstitial pneumonia is rare, what two conditions are associated with it?

A

HIV and Sjogren’s syndrome.

31
Q

What are the HRCT and histopathological findings of lymphoid interstitial pneumonia?

A

HRCT: ground glass and nodular infiltrates due to resolving cysts.

Histopathology: dense lymphocyte infiltrates with T-cells +++.

32
Q

What is CPFE (combined pulmonary fibrosis and empysema)?

How common is is it in the ILD (interstitial lung disease population)?

What other lung condition is an association of CPFE?

A

Interstitial lung disease + emphysema (obvious)

Found in 30-40%

Pulmonary HTN (cor pulmonale) is associated with CPFE.

33
Q

For CPFE (combined pulmonary fibrosis and emphysema), what are the finding upon:

  1. PFT
  2. HRCT
A
  1. PFT:
    - ‘normal’ lung volumes due to hyperinflation (emphysema) + restriction (ILD)
    - Marked decreased TLCO (gas transfer)
  2. HRCT:
    - Upper zone emphysema
    - Lower zone fibrosis (IPF/UIP)
34
Q

What are the CT-findings of pulmonary sarcoidosis (2)?

A

Upper +/- mid zone fibrosis (compare to lower zones in NSIP and UIP)

Bilateral symmetrical mediastinal lymphadenopathy.

35
Q

What are the histopathological findings of pulmonary sarcoidosis (2)?

A

Non-caseating granulomas

Normal lung parenchyma

36
Q

What is the treatment of pulmonary sarcoidosis?

If adequately treated, does it recur?

A

No treatment required unless symptomatic.

Steroids and methotrexate for progressive disease.

May recur after treatment.

37
Q

What is Lofgen sydrome?

What is the prognosis?

A

‘you can LAUGH at the SARcastic Attempt to Entertain us’

Lofgrens = Sarcoidosis + Arthralgia + Erythema nodosum

Good prognosis.

38
Q

What interstitial lung disease affect NON-smoking post-menopausal women with cysts in the kidney or ovaries?

What are the typical scenarios this condition should be considered as a DDx?

A

Pulmonary LAM (pulmonary lymphangio leiomyomatosis)

A young or middle aged woman with pneumothorax, chylothorax, and progressive shortness of breath without clear explanation.

A woman misdiagnosed with COPD despite having no history of smoking or asthma.

39
Q

Pulmonary LAM (pulmonary lymphangio leiomyomatosis) has what CT-findings?

A

CYSTs.

Also: hyperinflation and chylous (lymph) effusions.

40
Q

What is the treatment of pulmonary LAM (pulmonary lymphangio leiomyomatosis)?

A

Sirolimus (mTOR) inhibition) changes the progression of disease.

41
Q

What is the diagnostic criteria for OSA (obstructive sleep apnoea)?

How is the severity of OSA graded?

A

OSA = AHI > 5 + symptoms of OSA

AHI = apnoea-hypopnoea index (number of apnoea/hyponoea events per hour)

OSA is graded by AHI:
Mild: AHI 5-15
Moderare: AHI 15-30
Severe: AHI 30-120

42
Q

Compare and contrast VQ-scans and CTPA in the investigation of PE.

Which is preferred in pregnancy?

A

VQ-scan:

  • Sensitive (esp. small segmental PEs)
  • Better for chronic PE
  • Needs the CXR to be normal for interpretation.

CTPA:
- Specific

VC-scan is preferred in pregnancy.

43
Q

Compare sarcoidosis to TB in simple terms.

A

Sarcoidosis = non-necrotising (non-caseating) granulomas in multiple organs

TB = necrotising (caseating) granulomas

Use of necrotising is better than caseating in terms of precise description of microscopic appearance:

  • Necrotising granulomas suggest infective aetiology.
  • Non-necrotising granulomas suggest non-infectious aetiology (however may be found in infective TB cases on biopsy)
44
Q

What are some extra-pulmonary manifestations of sarcoidosis?

A

‘sarcoidosis scare nurses’ - SCARE RN:

Skin lesions

Calcium high (hypercalcaemia)

Arrhythmias

Reticuloendothelial system (mononuclear phagocyte system) enlargement: LNs (included parotid enlargement), hepatosplenomegaly.

Renal Impairment / CRF

Neurological (muscle weakness and nerve palsy)

45
Q

What are the biochemical features of sarcoidosis?

A

Elevated serum ACE (angiotensin converting enzyme) levels in 75% of cases.

Hypercalcaemia +/- renal stones

46
Q

Compare the serum calcium levels in sarcoidosis and secondary hyperparathyroidism.

A

Sarcoidosis = hypercalcaemia

Secondary hyperparathyroidism due to CRF = hypocalcaemia

47
Q

Regarding lung volumes, describe the component of:

  1. TLC (total lung volume)
  2. VC (vital capacity)
  3. IC (inspiratory capacity)
  4. FRC (functional residual capacity)
A

From top to bottom 4 components (ITER):

IRV (inspiratory reserve volume)
TV (tidal volume)
ERV (expiratory reserve volume)
RV (residual volume)

Now:
TLC (all) = IRV + TV + ERV + RV
VC (all except RV) = IRV + TV + ERV
IC (top bit) = IRV + TV
FRC (bottom bit) = ERV + RV

FRACP (20 decks)

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