Restrictive Pulmonary Disorders

Question 1

Lung restriction due to causes outwith the lungs eg.s

Answer 1

Skeletal - kyphoscoliosis, ankylosing spondylitis, rib #
Muscle weakness - myaesthenia gravis, Guillain-Barre
Obesity/ascites

Question 2

Rectrictive lung diseases affect lung ____

Answer 2

interstitium/parenchyma - tissue and space around alveoli

Question 3

effect of lung restriction arising outwith the lungs

Answer 3

alveolar under ventilation
low arterial Sa+PaO2 and raised PaCO2
reduced lung vol

Question 4

ILD overall effect

Answer 4

impaired alveolar gas exchange
low Pa+SaO2 and normal PaCO2
alveolar ventilation is normal

Question 5

6 broad causes of ILD

Answer 5

fluid
consolidation
inflammatory infiltrate
Dust
carcinomatosis
eosinophilic

Question 6

Causes of fluid in lung interstitium

Answer 6

LHF
altitude sickness
sepsis
ARDS

Question 7

Causes of consolidation of alveolar air spaces => ILD

Answer 7

pneumonia
infarction - PE/vasculitis
BOOP/COP - crytogenic organising pneumonia

Question 8

Causes of inflammatory infiltrate in alveolar walls (alveolitis -> ILD)

Answer 8

Granulomatous alveolitis - EAA, Sarcoidosis
Drug induced - amiodarone, bleomycin, methotrexate, gold
toxic fumes - Cl2
fibrosing alveolitis - rheumatoid, IPF
AI - SLE, Wegener’s, Churg-Strauss, Behcets

Question 9

Eg.s of pneumoconiosis - dust ILD

Answer 9

fibrogenic - asbestosis,silicosis

non-fibrinogenic - baritosis

Question 10

Spirometry pattern of restrictives

Answer 10

FEV1 reduced
FVC reduced
FEV1% and peak flow normal
DLCO reduced
Pa+SaO2 reduced

Question 11

___ and ____ raised in sarcoidosis

Answer 11

serum ACE and Ca2+

Question 12

Important Hx points in ILD

Answer 12

pets, drugs, occupation, arthritis

Question 13

Tests for ILD

Answer 13

Spirometry
Bloods - antibodies, serum ACE, Ca2+
CXR - LV hypertrophy, bilateral infiltrates
ECG - LHF
CT - inflam = groundglass ; fibrotic nodules
BAL/sputum
biopsy

Question 14

Treatments for ILD

Answer 14

1- remove trigger
Immunosuppressants:
2 - systemic corticosteroids
3 - PO azathioprine, anti-fibrotics (pirfenidone, nintendanib) + anti-ox (acetylcysteine) for IPF
O2 if needed
4- transplant

Question 15

EAA/_____ is a ___ hypersensitivity reaction

eg.s

Answer 15

hypersensitivity pneumonitis
Type 3 - immune complex
Farmer’s/pigeon fancier’s lung

Question 16

Sarcoidosis is more/less common in smokers

Answer 16

less

Question 17

sarcoidosis commonly affects

Answer 17

lungs, lymph nodes, joints, liver, skin, eyes

Question 18

acute sarcoidosis signs and symptoms

treatment

Answer 18

erythema nodosum, bilateral hilar lymphadenopathy, arthritis, uveitis, parotitis, fever
NO TREATMENT

Question 19

chronic sarcoidosis signs and symptoms

treatment

Answer 19

alveolitis, skin infiltrations, peripheral lymphadenopathy, hypercalcaemia
Oral steroids, immunosuppression (anti-TNF, azathioprine, methotrexate)

Question 20

Diagnosis of Sarcoidosis tests

Answer 20

CXR = bilateral hilar lymphadenopathy
CT = peripheral nodular infiltrate
Biopsy = non-casseating granuloma
Bloods - increased serum ACE (activity marker) and Ca2+, inflammatory markers

Question 21

2ndry causes of pulmonary fibrosis -

Answer 21

rheumatoid, SLE, systemic sclerosis, asbestos, drugs

Question 22

IPF presentation:

Answer 22

progressive SOB, dry cough, clubbing, bilateral fine inspiratory crackles, PFT restrictive pattern

Question 23

IPF imaging:

Answer 23

CXR bilateral infiltrates

CT reticulonodular fibrotic change worse at bases

Question 24

Drugs to slow progression of IPF

Answer 24

N-acetyl cisteine has anti-fibrotic effects

Question 25

Caplan’s syndrome =

Answer 25

rheumatoid pneumoconiosis - pulmonary nodules

coal workers get it

Question 26

CXR shows egg-shell calcification of hilar lymph nodes

Answer 26

Silicosis

Question 27

Asbestosis causes ___ and a ___ PFT pattern

Answer 27

diffuse pleural thickening

restrictive

Question 28

IPF fibrosis pattern:

Answer 28

subpleural and basal fibrosis

lung structure replaced by dilated spaces with fibrous walls and splints lung so it can’t expand

Question 29

____ asbestos fibres are more dangerous than ___ fibres

Answer 29

amphibole (straight) worse than serpentine (curved)