Respiratory MCQs

Question 1

What is the best measure of clinical progression in Interstitial pulmonary fibrosis ?

Answer 1

FVC in lung function testing

Question 2

In patients with apical emphysema but basal fibrosis what pattern would we see on spirometry ?

Answer 2

May have a normal loop but a majorly impaired diffusion capacity

Question 3

Radiological features of definite usual interstitial pneumonia

Answer 3

Honeycombing, traction bronchiectasis and reticulation

Architectural distortion

Question 4

How to confirm diagnosis of usual interstitial pneumonia?

Answer 4

If HRCT displays features, there is no need for biopsy

Question 5

What are the names of anti-fibrotics approved for use in Australia

Answer 5

Nintedanib and Pirfenadone

Question 6

Bronchodialator respose required in spirometry for dx. asthma

Answer 6

> 12% and >200mls

Question 7

Name drug used and guide used in prevocation testing

Answer 7

Stop the challenge after the patient drops their FEV1 >20% - then you check the dose of methacholine that is required to give them this drop by >20% (PD20 provocation dose) = the smaller the number the more sensitive their airways are.
<8mg/ml is diagnostic

Question 8

Indirect provocation testing

Answer 8

Detects the presence of inflammatory cells in the airways and is more indicative of current asthma (than direct method)
Positive if > or = 15% reduction in FEV1
More specific but less sensitive than direct challenge
Mannitol, exercise, hypertonic saline all used

Question 9

Omalizumab MOA

Answer 9

Recombinant, DNA derived, monoclonal anti-IgE antibody
Binds circulating IgE and limits allergic response
Side effects
Mainly nasopharyngitis (28%), URTIs 16%), headaches
- Patients need to cary epipens ?

Only used as last line- FEV1 less than 80 - high IgE

Question 10

Mepolizumab MOA

Answer 10

Monoclonal antibody against interleukin 5

Eosinophilia

Question 11

Two types of asthma

Answer 11

Atopic and non-atopic. Both caused by IgE production. However atopic asthma is systemic IgE whereas non-atopic is local IgE production

Question 12

Histological changes in asthma

Answer 12

Increased goblet cells and mucous production in pseudostratified columnar epithelium, thickened basement membrane, increased histamine from mast cells in the lamina propria as well as increased neutrophils and helper T cells. Smooth muscle hypertrophy is also present.

Question 13

Pathophysiology of atopic asthma

Answer 13

Allergen inhaled –> taken up by columnar cells –> thymic stromal lymphocytes secreted –> activated dendritic cells in the lamina propria produce chemo-attractant conditioned to attract T-2 helper T cells –> activated t-helper 2 cells stimulate plasma cells via IL13 and IL4 promoting IgE production in plasma cells –> T helper 2 also produce IL9 to stimulate mast cells –> finally IL5 prodiuced by helper T 2 cells stimulates eosinophil production from the bone marrow

IgE also attaches to mast cells to produce histamine, leukotrienes and prostaglandins which in turn activates smooth muscle

Stem cell factor produced by columnar cells maintains mast cells in the area

T helper 1 - normally found in lungs
T helper 2 cells, not normally found in the lungs are upregulated and systemically cause an overproduction of antibodies.

Question 14

How to assess severity of asthma on presentation

Answer 14

Mild/moderate –> able to speak whole sentences in one breath
Severe –> any of: unable to speak in sentences, visibly breathless, sats 90-94% or increased work of breathing
Life threatening –> drowsy, collapsed, exhausted, cyanosed, sats less than 90, poor inspiratory effort

Question 15

Management of asthma by severity

Answer 15

Mild-moderate 4-12 puffs of salbutamol via MDI (100 microg/puff)
Severe –> 12 puffs of salbutamol (100 microg/puff) every 20 mins for 1 hour (i.e. 3 cycles) and 8 x puffs of ipatropium bromide (21 microg/actuation)
OR
nebs: 5 mg salbutamol and 500 microg ipatropium
drive with air is possible, but other wise oxygen, then commence oxygen post.
AIM 93-95% SATS
Life threatening –> continuous 5mg salbutamol nebs prepare to intubate or ventilate

REASSESS

Question 16

After first reassessment in asthma what is the next management step ?

Answer 16

If poor response at any level –> Magnesium sulphate 10 mmol over 20 mins
If no improvement or worsening –> corticosteroids
Oral pred 37.5 mg -50mg for 5-10 days. If oral not available then IV Hydrocortisone 100 mg 6 hrly.
Post 1 hour of treatment REASSESS and transfer home vs. ward vs. ICU depending on treatment requirements

Question 17

Usual interstitial pneumonia (UIP)

Answer 17

Subpleural reticulation, apical basal gradient, traction bronchiectasis, honeycombing

Question 18

Non-specific interstitial pneumonia (NSIP)

Answer 18

Ground glass change, apical to basal gradient, subpleural sparing, traction bronchiectasis

Question 19

Organising pneumonia (OP)

Answer 19

Consolidation, bronchovascular in distribution

Question 20

Desquamative interstitial pneumonia

Answer 20

Ground glass change with small cysts

Question 21

Respiratory bronchiolitis interstital lung disease

Answer 21

Centrilobular ground glass nodularity

Question 22

Lymphocytic interstital pneumonia

Answer 22

Ground glass changes with cysts

Question 23

Hypersensitivity pneumonitis

Answer 23

Acute: Ground glass nodularity
Chronic: Fibrotic changes akin to UIP but with mosaicism, ground glass and gas trapping

Question 24

Description of terms used such as “UIP”, DIP, NSIP

Answer 24

These are radiological terms use in lung disease to describe the pattern of disease, however lots of diseases can fit the single pattern

Question 25

Pattern seen in Idiopathic pulmonary fibrosis and treatment

Answer 25

UIP pattern of disease

Anti-fibrinolytics: Pirfenidone or nintedanib

Question 26

Describe IPF

Answer 26

Chronic inexorably progressive fibrosing lung disease of unknown cause.
Other causes of fibrosing disease need to be excluded
Radiological UIP is a strong surrogate for histology - lung biopsy seldom needed.

Typical patient: older man in their 6-7th decade, past smoking history, GORD, family history.

Question 27

Unlikely patients to have IPF

Answer 27

Exposure to known HP antigen, Squarks on auscultation, rheumatological signs, pneumotoxic drugs

Question 28

pirfenidone MOA

Answer 28

ACTs through TGF-B to reduce fibroblast proliferation
Slows rate of disease progression.
Improves survival
Photosens. rash and GIT upset

Question 29

Nintedanib MOA

Answer 29

Inhibits multiple Tyrosine kinases - slows disease progress. Major SE diarrhoea and weight loss. Small bleeding risk - avoid if anticoag,.
Improved progress in Systemic sclerosis too

Question 30

Genes associated with Pulmonary fibrosis

Answer 30

TERT, TERC, RTEL1, PARN

associated with disease anticipation

Question 31

Association with telomerase complex genetic mutation

Answer 31

IPF and dyskeratosis congentia - leading to aplastic anaemia. When inherited in child, anticipation is noted.
Autosomal dominant inheritance

Question 32

Clinical features of telemere shortening

Answer 32

Affects any rapid turn over cells - i.e. greying of hair, liver cirrhosis, opportunistic infection, emphysema, osteoporosis, coronary artery disease

Question 33

Gene mutation associated with RA-ILD and Pulm. fibrosis

Answer 33

MUC5B ~50% of ILD patients

Question 34

Which immmunosuppressant is contraindicated in SS related ILD

Answer 34

Methotrexate - can cause ILD

Question 35

Which immunosuppression is contraindicated in IPF

Answer 35

Aza and mycophenolate

Question 36

Antibody assicated with Dermatomyositis and lung disease

Answer 36

anti-MDA5, often have negative myopathic screen- pneumomediastinum is the most common presentation in this micro-population

Question 37

Differentiated Langerhans cell histiocytosis vs. LAM

Answer 37

Langerhans - irregularly shaped cysts and sparing of the costophrenic recess. Langerhans cells are dendritic cells
LAM cysts are typically found in the costophrenic recess ‘LAMbsall to the gullies’. Typically smooth cysts.
(LAM = lymphangioleiomyomatosis)

Question 38

Drugs that cause pulmonary fibrosis

Answer 38

BackCountry MAN
Biologics
Chemo

Methotrexate
Amiodarone
Nitrofuantoin

Question 39

CVID classic presentation

Answer 39

Recurrent sinusitis
Associated with Granulomatous lymphocytic interstitial lung disease GLILD can mimic sarcoidosis in producing ACE
Diagnosed with Bronchioalveolar lavage

Question 40

LAM classic presentation

Answer 40

Pulmonary cysts and renal angiomyolipoma in a young woman.
There may also be sporadic tuberous sclerosis associated
High VEG-F levels may assist with dx.
Management is with Rapamycin (Sirolimus) an mTOR inhibitor

Question 41

Pulmonary alveolar proteinosis presentation

Answer 41

Chronic cough and dyspnoea - crazy paving on CT in lower zones. Milky fluid on BAL with periodic acid Schiff positivity. Anti-GM-CSF antibody is expected.
whole lung lavage, immunomodulation and inhaled GM-CSF management.

Question 42

At which level of flow volume loop is the pulmonary vascular resistance at its lowest ?

Answer 42

FRC (Functional residual capacity)

I.e. the bottom of the tidal volume in normal respiration

Question 43

Starlings law and how it relates to pulmonary circulation

Answer 43

Fluid movement out of capillary by the difference in hydrostatic and oncotic gradients. Oncotic gradient in pulmonary physiology is low but favours reabsorption. Hydrostatic pressure is NIL. Therefor a slight increase in pulmonary pressure leads to large volume extravasation.
Thin walled vessels without much smooth muscle means that vessels are highly compliant and accomodate large volumes with small pressure changes

Question 44

Lung volumes affect on PVR

Answer 44

Large extra-alveolar vessel pressure decreases as the vessels are pulled open at large pulmonary volumes, small vessels are compressed and pressure increases.
At small lung volumes the opposite occurs.

Question 45

Sarcoidosis syndromes

Answer 45

Loftgrens: Bilateral hilar lymphadenopathy, erythema nodosum, fever and polyarthralgia
Mikulicz syndrome: enlargement of the parotic and lacrimal glans
Heerfordts: Parotid enlargement, fever, uveitis and hilar involvement. Occasionally facial nerve palsy

All typified by non-caseating granulomas with hypercalcaemia (Macrophages inside granulomas cause an increased conversion of Vit D to 1,25 dihydroxycholecalciferol