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Respiratory Flashcards

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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)?

Study These Flashcards
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)?

Study These Flashcards
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.

Study These Flashcards
A

False.

Mostly collagen, inflammatory cells are minimal.

22
Q

What are the 7 types of idiopathic interstitial pneumonia?

Study These Flashcards
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.

Study These Flashcards
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
Which 2 features differentiates UIP from NSIP on HRCT?
UIP: honeycombing but NO ground glass NSIP: ground glass but NO honeycombing Remember: hUney/UIP and grouNd/NSIP
26
True/False: NSIP, similar to UIP, is also basal/subpleural sparing of the apices, symmetrical, with traction bronchiectasis and a reticular patient.
True.
27
What is the histopathology of NSIP (2)?
1. Inflammatory cells with ABSENCE of fibroblastic foci. | 2. Homogenous pattern and not patchy
28
What is NSIP (non-specific interstitial pneumonia)?
Mainly connective tissue related lung fibrosis, may also be idiopathic. Spectrum of fibrosis vs. ground glass (think inflammatory) changes.
29
True/False: medical therapy is NOT effective in NSIP (non-specific interstitial pneumonia)?
False. NSIP (unlike IPF/UIP) may be responsive to prednisolone/cyclophosphamise (if inflammation > fibrosis)
30
Lymphoid interstitial pneumonia is rare, what two conditions are associated with it?
HIV and Sjogren's syndrome.
31
What are the HRCT and histopathological findings of lymphoid interstitial pneumonia?
HRCT: ground glass and nodular infiltrates due to resolving cysts. Histopathology: dense lymphocyte infiltrates with T-cells +++.
32
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?
Interstitial lung disease + emphysema (obvious) Found in 30-40% Pulmonary HTN (cor pulmonale) is associated with CPFE.
33
For CPFE (combined pulmonary fibrosis and emphysema), what are the finding upon: 1. PFT 2. HRCT
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
What are the CT-findings of pulmonary sarcoidosis (2)?
Upper +/- mid zone fibrosis (compare to lower zones in NSIP and UIP) Bilateral symmetrical mediastinal lymphadenopathy.
35
What are the histopathological findings of pulmonary sarcoidosis (2)?
Non-caseating granulomas Normal lung parenchyma
36
What is the treatment of pulmonary sarcoidosis? If adequately treated, does it recur?
No treatment required unless symptomatic. Steroids and methotrexate for progressive disease. May recur after treatment.
37
What is Lofgen sydrome? What is the prognosis?
'you can LAUGH at the SARcastic Attempt to Entertain us' Lofgrens = Sarcoidosis + Arthralgia + Erythema nodosum Good prognosis.
38
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?
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
Pulmonary LAM (pulmonary lymphangio leiomyomatosis) has what CT-findings?
CYSTs. Also: hyperinflation and chylous (lymph) effusions.
40
What is the treatment of pulmonary LAM (pulmonary lymphangio leiomyomatosis)?
Sirolimus (mTOR) inhibition) changes the progression of disease.
41
What is the diagnostic criteria for OSA (obstructive sleep apnoea)? How is the severity of OSA graded?
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
Compare and contrast VQ-scans and CTPA in the investigation of PE. Which is preferred in pregnancy?
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
Compare sarcoidosis to TB in simple terms.
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
What are some extra-pulmonary manifestations of sarcoidosis?
'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
What are the biochemical features of sarcoidosis?
Elevated serum ACE (angiotensin converting enzyme) levels in 75% of cases. Hypercalcaemia +/- renal stones
46
Compare the serum calcium levels in sarcoidosis and secondary hyperparathyroidism.
Sarcoidosis = hypercalcaemia Secondary hyperparathyroidism due to CRF = hypocalcaemia
47
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)
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 ```

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