Resp

Question 1

Pulm fibrosis Ix

Answer 1

-Obs + ABG
- Bloods: fbc, U+E, lft, crp + ANA,ANCA,RF,antiCCP, dsDNA, Anti-Scl-70
-Sputum culture
-CXR (reticulonodular change) –> HRCT

-Pulmonary function test:
—> restrictive: FEV1/FVC >0.8
very reduced FVC,
Low transfer transfer factor
KCO low

Question 2

Lower zone fibrosis

Answer 2

CTD - Lupus, RA, scleroderma
Asbestosis
Idiopathic - more likely clubbing - UIP
Drugs: MTX, amiodarone (AF), nitrofurantoin

Question 3

Upper zone fibrosis

Answer 3

Hypersensitivity pneumonitis
Ank spond
Sarcoidosis
ABPA
Old TB
Langerhans cell histiocytosis

Question 4

HRCT findings with pulmonary fibrosis

Answer 4

UIP = honeycombing - more often IPF
NSIP (non-specific) = ground glass - more often assoc CTD/AI disease

Question 5

Treatment pul fibrosis

Answer 5

vaccine, smoking cessation, pul rehab,
treating course - CTD = immunosuppressive (steroids or steroid sparing)

if IPF - specialist centre to consider anti-fibrotic (pirfendidone ipf only)

Nintedanib can be used in other causes - strict criteria FEV1 50-80%

Single lung transplant

Question 6

Prognosis of PF based on CT

Answer 6

GG changes - 80 % respond to immunosuppression

Honeycomb - 80% no response to immunosuppression

Question 7

Pulmonary HTN causes

Answer 7

WHO clinical classification:

1) Pulmonary arterial hypertension - idiopathic
secondary: CTD, congenital heart disease, HIV, drugs/toxins

2) Left heart disease: function/valves

3) Lung disease: COPD, ILD, sarcoid

4) Chronic thromboembolic disease

5) Miscellaneous: long-term HD

Question 8

Treatment for pulmonary hypertension

Answer 8

Based on cause:
1) PAH: pulmonary vasodilators - prostacyclin analogues, endothelin receptor antagonists, sildenaifl

4) lifelong anti coag

2,3,5) underlying cause

Question 9

Investigation Pulm HTN

Answer 9

Fbc (anaemia), RFx, LFT (porto-pulmonary PH), HIV, autoimmune (SLE, scleroderma), TFT

Echo - RV size/function, estimate pulm pressure if TR, left sided disease re cause

Imaging: CTPA - if thromboembolic

Lung function tests - lung causes ILD/COPD

Dx: right heart catheterisation = raised pulmonary pressure

Question 10

Complications pulm htn

Answer 10

R heart failure
Atrial arrhythmia
Risk of death in pregnancy (pulmonary blood flow increases significantly)