Respiratory

Question 1

What is Ivacaftor? What is it used for?

Answer 1

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

Question 2

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

Answer 2

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

Question 3

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

Answer 3

x4

Question 4

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

Answer 4

Clinical trials.

Closely monitor PFT - transplant if TLCO = 40%

Question 5

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?

Answer 5

When TLCO = 40%

Single lung transplants are preferable

Age cut-off is 65 yrs

Question 6

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

Steroids
Immunomodulators
Anti-fibrotics
NAC
Warfarin

Answer 6

None.

Question 7

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

Answer 7

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

Question 8

What is another name for Constrictive Bronchiolitis?

Answer 8

Bronchiolitis Obliterans

Question 9

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

Answer 9

Many.

Cryptogenic

OR

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

Question 10

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?

Answer 10

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.

Question 11

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

Answer 11

True.

Question 12

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

Answer 12

Idiopathic

OR

Non-specific response to infection/drugs

Question 13

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

Answer 13

Mild-moderate restriction.

Reduced TLCO

Question 14

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

Answer 14

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

Question 15

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

Answer 15

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).

Question 16

What is best measure of deterioration in IPF?

Answer 16

10% reduction in FVC on PFT

(previously: 15% reduction in DLCO)

Desaturation only occurs in advanced disease, therefore not useful.

Question 17

Give 2 biomarkers of poor prognosis in IPF/UIP.

Answer 17

1. Circulating fibrocytes suggests poor prognosis in IPF

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

Question 18

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

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

Answer 18

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.

Question 19

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

Answer 19

PHaF-BRATS (8):

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

Question 20

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

Answer 20

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

Question 21

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

Answer 21

False.

Mostly collagen, inflammatory cells are minimal.

Question 22

What are the 7 types of idiopathic interstitial pneumonia?

Answer 22

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)

Question 23

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

Answer 23

False.

Ground glass changes present but honeycombing is absent.

Question 24

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

Answer 24

True.

Question 25

Which 2 features differentiates UIP from NSIP on HRCT?

Answer 25

UIP: honeycombing but NO ground glass NSIP: ground glass but NO honeycombing Remember: hUney/UIP and grouNd/NSIP

Question 26

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

Answer 26

True.

Question 27

What is the histopathology of NSIP (2)?

Answer 27

1. Inflammatory cells with ABSENCE of fibroblastic foci. | 2. Homogenous pattern and not patchy

Question 28

What is NSIP (non-specific interstitial pneumonia)?

Answer 28

Mainly connective tissue related lung fibrosis, may also be idiopathic. Spectrum of fibrosis vs. ground glass (think inflammatory) changes.

Question 29

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

Answer 29

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

Question 30

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

Answer 30

HIV and Sjogren's syndrome.

Question 31

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

Answer 31

HRCT: ground glass and nodular infiltrates due to resolving cysts. Histopathology: dense lymphocyte infiltrates with T-cells +++.

Question 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?

Answer 32

Interstitial lung disease + emphysema (obvious) Found in 30-40% Pulmonary HTN (cor pulmonale) is associated with CPFE.

Question 33

For CPFE (combined pulmonary fibrosis and emphysema), what are the finding upon: 1. PFT 2. HRCT

Answer 33

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)

Question 34

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

Answer 34

Upper +/- mid zone fibrosis (compare to lower zones in NSIP and UIP) Bilateral symmetrical mediastinal lymphadenopathy.

Question 35

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

Answer 35

Non-caseating granulomas Normal lung parenchyma

Question 36

What is the treatment of pulmonary sarcoidosis? If adequately treated, does it recur?

Answer 36

No treatment required unless symptomatic. Steroids and methotrexate for progressive disease. May recur after treatment.

Question 37

What is Lofgen sydrome? What is the prognosis?

Answer 37

'you can LAUGH at the SARcastic Attempt to Entertain us' Lofgrens = Sarcoidosis + Arthralgia + Erythema nodosum Good prognosis.

Question 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?

Answer 38

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.

Question 39

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

Answer 39

CYSTs. Also: hyperinflation and chylous (lymph) effusions.

Question 40

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

Answer 40

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

Question 41

What is the diagnostic criteria for OSA (obstructive sleep apnoea)? How is the severity of OSA graded?

Answer 41

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

Question 42

Compare and contrast VQ-scans and CTPA in the investigation of PE. Which is preferred in pregnancy?

Answer 42

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.

Question 43

Compare sarcoidosis to TB in simple terms.

Answer 43

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)

Question 44

What are some extra-pulmonary manifestations of sarcoidosis?

Answer 44

'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)

Question 45

What are the biochemical features of sarcoidosis?

Answer 45

Elevated serum ACE (angiotensin converting enzyme) levels in 75% of cases. Hypercalcaemia +/- renal stones

Question 46

Compare the serum calcium levels in sarcoidosis and secondary hyperparathyroidism.

Answer 46

Sarcoidosis = hypercalcaemia Secondary hyperparathyroidism due to CRF = hypocalcaemia

Question 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)

Answer 47

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 ```