Interstitial lung disease

Question 1

what is the condition of undifferentiated connective tissue disease when associated with pulm fibrosis?

Answer 1

About 25% of patients with features of systemic autoimmune disease do not fulfil ACR classification criteria for CTD.

• 65-94% do not develop into a ‘differentiated’ CTD
• Criteria – signs and symptoms of CTD, positive auto-Abs, duration >1yr

•Most often display NSIP pattern on biopsy

these patients respond different to IPF, and thus are treated differently

Question 2

there are a few familial interstitial pneumonias associated with particular mutations.

Any idea what they are? or even, what they are about?

Answer 2

surfactant related

MUCB - promoter region mutation

telomer/telomerase mutations

Question 3

any idea which of the ILDs correlate HRCT findings and RFTs?

Answer 3

IPF - FVC, DLco and PaO2 with HRCT

sarcoid - fvc, DLco, dyspnoea correlate with HRCT

rheumatoid lung - DLco with HRCT

Scleroderma assoc ILD - DLco strongly assoc with HRCT and also SpO2 on exercise

NSIP - FVC with HRCT

Question 4

which of the ILDs have the worst prognosis?

Answer 4

IPF is pretty bad

the median survival is about 3 years.

Question 5

where is the changes in the HRCT in IPF?

Answer 5

sub pleural and bibasal

the characteristics are honeycombing and ground glass

Question 6

what is the difference between IPF and NSIP on HRCT?

Answer 6

the major change is the NSIP doesn’t have honeycombing in the early stages

it still has the ground glass bibasal changes though

also - histopathologically the changes are more homogenous, as opposed to IPF which has a lot of heterogeneity about it

Question 7

where is the radiological distribution of hypersensitivity pneumonitis?

Answer 7

it is predominantly upper lobe with ground glass, reticular

it is broncho-centric - because it is associated with some sort of antigen

it is NOT a bibasal subpleural predominance

Question 8

what is the clinical presentation of Langerhans histiocytosis?

Answer 8

this is a disease of young adults, usually current smokers

their scans look horrible. they have these weird stellate lesions with large irregular “weird” cysts

it has a slight upper lobe preference

you can trial ‘roids. patient MUST STOP SMOKING

if you transplant, can still recur many years later

can be systemic with lesions in bones and all kinds of places.

Question 9

what are the findings of respiratory bronchiolitis ILD?

Answer 9

this is a broncho-centric lung disease with predilection for the upper lobes

this is highly associated with smokers

it is associated with a accumulation of macrophages

there is emphysema and air trapping as well

it is not subpleural

RFTs can even look obstructive

Question 10

where does desquamative interestitial pneumonitis primarily affect? (what does it look like on HRCT?)

Answer 10

this can be a smokers condition

can occur with haem malignancies

prognosis is okay 70% at 10 years

ct is ground glass - lots of involved macrophages

typically lower zone and peripheral

probably DIP and RB-ILD are overlap conditions

Question 11

what is the clinical presentation of cryptogenic organising pneumonia

Answer 11

this is a steroid responsive condition

the ct can look like anything but classically is pneumonia like with consolidation, and as an extension of this, is broncho-centric.

presents with fever and dyspnoea

can be a complication of RA or post drug, like amiodarone - although, cryptogenic means known. so if it’s 2nd to amiodarone, it should be called organising pneumonia secondary to amiodarone

the reverse Atoll sign is heavily associated with this condition - it is consolidation surrounded by a ring of denser consolidation

Question 12

does lung function studies provide treatment or prognostic information in UIP/NSIP?

any other conditions this applies to?

Answer 12

Significant decline in FVC + DLCO at 6 predicts mortality as strongly as histological diagnosis

•Significant decline in FVC + DLCO at 12 months predicts mortality more strongly than baseline histological diagnosis i.e. UIP vs NSIP provides no additional prognostic information once serial PFTs are considered

Significant change in FVC and DLCO are predictive of survival in SSc-ILD

Significant change in FVC is predictive of survival in EAA

Question 13

what is perfinidone?

Answer 13

this is an anti fibrotic agent that has been approved for use in asia.

it is indicated for IPF

in US and australia still pending this clearance.

there was some decline, but it basically held the disease statically

Question 14

any role for warfarin in IPF?

Answer 14

nope. not purely for this indication

studies have not supported this. (there was initial support in 2005)

Question 15

is there any role for PPI in IPF?

Answer 15

surprisingly yes.

particularly things like funduplication too

Question 16

any role for rehab in IPF?

Answer 16

yep - pulm rehab should be referred to.

there was a demonstrated improvement in 6mwd and dyspnoea following this.

Question 17

how should we treat IPF?

Answer 17

at present: NAC and pred with pirfenidone once it becomes available

anticoagulation is not really supported any more for treatment.

sildenafil is still a MAYBE

Question 18

in SSc-ILD what are the agents?

Answer 18

cyclophosphamide or mycophenolate are where the evidence is

realistically cyclophosphamide will be the exam answer.

it also impacts the skin signs