Interstitial lung disease

Question 1

What is ILD?

Answer 1

lung disease associated with interstitium , tissue around the alveoli

Question 2

what is the most common ILD type?

Answer 2

idiopathic pulmonary fibrosis

others: systemic sclerosis, Lupus

Question 3

what is idiopathic pulmonary fibrosis?

Answer 3

progressive scarring and fibrosis of the lung interstitium

no discernible underlying cause

Question 4

what drugs (name 3 ) that can cause IPF?

Answer 4

methotrexate, amiodarone, bleomycin

Question 5

IPF is common over what age group?

Answer 5

> 60 yrs

Question 6

Is there ethnic factors involves in IPF?

Answer 6

no, but a familial variant (FPF, Familial Pulmonary Fibrosis) exists

Question 7

What are the clinical features of IPF?

Answer 7

seen at an older age
Progressive dyspnoea worse on exertion
cough
finger clubing (may be seen)
>bilateral inspiratory crackles<
restrictive pattern on spirometry

Question 8

Investigation of IPF

Answer 8

examination and history
Peak flow test
DLco (reduced)
CXR (bilateral lower zone reticulonodular shadows)
HRCT= round glass appearance

Reticulation (net like) results from thickening of the interlobular or intralobular septa and appears as several linear opacities that resemble a mesh or a net on HRCT scans.

Reticular= too many lines
nodular=too many dots

Question 9

management of IPF (idiopathic pulmonary fibrosis)?

Answer 9

pulmonary rehabilitation
supportive care
guidelines recommend the use tyrosine kinase inhibitotrs or pirfenidone

average life expectancy is 3-5 years from the time of diagnosis

Question 10

What is sarcoidosis

Answer 10

chronic granulomatous disorder of unknown cause

multi-organ system disorder

histologically=non-caseating granulomas

Question 11

Who are the most commonly affected by sarcoidosis?

Answer 11

Higher incidence in Afro-Caribbean and
Scandinavian populations

Traditionally affects people in their second to
fourth decade

Question 12

Clinical features of sarcoidosis

Answer 12

Flu like illness-pyrexial
dry cough
cutaneous changes=erythema nodosum
maculopapular=both flat and raised rash
Anterior uveitis (inflammation of the middle layer), dry eyes
Hypercalcaemia (from granulomatous change in the disease, due to macrophages)

Lupus pernio=raised
indurated lesions on the cheeks, nose, ears
and forehead, may also be observed in
sarcoidosis

Question 13

What is Lofgren syndrome

Answer 13

fever
erythema nodosum
bilateral hilar lymphadenopathy
polyarthralgia.

This is an acute presentation of sarcoidosis and has a
good prognosis. Treat with bed rest and NSAIDs.

Question 14

Investigation of sarcoidosis

Answer 14

CXR
Bloods: calcium, ESR, LFT
spirometry
tuberculin skin test=negative (Mantoux test, used for TB diagnosis)

Question 15

Management of sarcoidosis

Answer 15

1. asymptomatic with bilateral lymphadenopathy=observe
2. symptomatic stage 2,3,5=oral/inhaled corticosteroid. Cytotoxics (methotrexate or hydroxychloroquine) if sever or unresponsive
3. Topical corticosteroid for cutaneous manifestation

mild=Hydrocortisone
Moderate=Eumovate
Potent=Betnovate
Potent with antimicrobials=fucibet

Question 16

Stage 1 sarcoidosis CXR findings

Answer 16

bilateral hilar lymphadenopathy

Question 17

Stage 2 sarcoidosis CXR findings

Answer 17

bihilar lymphadenopathy and reticulonodular infiltrates of parts or the entire lung.

Question 18

Stage 3 sarcoidosis CXR findings

Answer 18

bilateral pulmonary infiltrates without signs of hilar lymphadenopathy

Question 19

Stage 4 sarcoidosis CXR findings

Answer 19

fibrosis

Question 20

What is acute respiratory distress syndrome (ARDS)

Answer 20

refers to non-cardiogenic
pulmonary oedema and lung inflammation that leads
to severe respiratory difficulty

Question 21

what is a cause of ARDS

Answer 21

Sepsis
aspiration
pancreatitis
DIC

though to occur due to leakage of protein-rich fluid into alveolar–>inflammation–>hypoxia–>respiratory distress

Question 22

What is the diagnostic criteria of ARDS (Berlin criteria)

Answer 22

1. Dyspnoea and ARDS is acute
2. Bilateral infiltrates on CXR
3. PaO2:FiO2 ratio
   201–300 (mild), 200–100 (moderate), <100
   (severe)
4. Non-cardiogenic pulmonary oedema
5. Pulmonary wedge pressure (PWP)= a measure of left arterial pressure.

An elevated PWP suggests
cardiogenic pulmonary oedema, while a lower
PCWP may help indicate ARDS.

Question 23

Investigation in ARDS

Answer 23

CXR
Blood/sputum sample
urine culture (Sepsis)
echocardiogram (normal in ARDS)

Question 24

What is PCWP used to determine. as in gold standard

Answer 24

PCWP is considered the gold standard for determining the cause of acute pulmonary edema; this is likely to be present at a PWP of >20mmHg.

Question 25

what is the normal range of PCWP?

Answer 25

2-16mmHg

Question 26

management of ARDS

Answer 26

low tidal volume ventilation proning the patient, alters chest wall mechanics sedation to minimise patient/ventilator dyssynchrony, especially in those with hypoxia.

Question 27

what is extrinsic allergic alveolitis -EAA (hypersensitivity pneumonitis)

Answer 27

EAA is granulomatous inflammation occurs in the lung due to inhaling organic or chemical antigens or proteins.

Question 28

what is are the different types of extrinsic allergic alveolitis

Answer 28

Farmer's lung-mouldy hay-Saccharopolyspora rectivirgula Bird fancier's lung-protein in bird droppings Malt worker's lung- mouldy malt-Aspergillus clavatus Mushroom worker's lung

Question 29

Clinical features of EAA

Answer 29

expectorant cough SOB May present up to 8 hours after exposure recover within a week.

Question 30

Management of EAA

Answer 30

supportive-most resolve within a week remove offending antigen, change of occupation

Question 31

What is pneumoconiosis? coniosis=Any of various diseases or pathological conditions caused by dust

Answer 31

Coal workers lung. it is exemplified by fibrosis that occurs secondary to repeated inflammation caused by silica

Question 32

Which occupation causes silicosis?

Answer 32

bricklaying, tunnelling, pottery and ceramic i

Question 33

What is the difference between the cause of pneumoconiosis and silicosis?

Answer 33

pneumoconiosis= coal worker silicosis= can by due to coal work but also bricklaying , pottery and ceramic.

Question 34

What is Caplan syndrome

Answer 34

Coal worker’s pneumoconiosis with | associated rheumatoid arthritis

Question 35

management of pneumoconiosis

Answer 35

Both social and emotional support should be given to these patients. Coal worker’s pneumoconiosis is a notifiable disease. Silicosis and pneumoconiosis unfortunately have no specific therapy, but symptomatic management should be offered.

Question 36

Is pneumoconiosis and silicosis notifiable?

Answer 36

YES

Question 37

What occupations are associated with asbestos?

Answer 37

work at shipyards, train lines and in the plumbing industries Asbestosis

Question 38

What can exposure of asbestos cause?

Answer 38

lung cancer, specifically mesothelioma (cancer of the pleura)

Question 39

management of asbestosis

Answer 39

There is no specific therapy available. Treatment should focus on minimising further exposure to asbestos, as well as smoking cessation and managing any other co-morbid lung disease.

Question 40

What is the difference between asbestosis and mesothelioma?

Answer 40

Asbestosis and mesothelioma are both diseases caused by asbestos exposure, but they are not the same. The primary difference is that asbestosis is not cancerous and is limited to the lungs and respiratory tract. Mesothelioma is an incurable cancer that develops in mesothelial tissue, typically in the lungs and abdomen.

Question 41

investigation of asbestos related interstitial lung diseas?

Answer 41

CXR CT chest (high res, contrast) pleural biopsy

Question 42

Management of both asbestosis and mesothelioma?

Answer 42

Treatment is supportive, with pleural surgery being indicated for palliative symptomatic relief Patients who develop asbestos-related disease may be eligible for financial compensation, and it may be helpful to discuss this with them.