L12 - therapies in interstitial lung diseases

Question 1

IPD median survival

Answer 1

2-5 years from diagnosis

Question 2

IPF cause

Answer 2

hypothesised it was a disease of inflammation leading to scarring so originally immunosuppression was the mainstay of treatment

Question 3

IPF and immunosuppression drugs

Answer 3

actually increased mortality rate

Question 4

Pirfenidone

Answer 4

IPF new treamtnet
targets fibroblasts by preventing TGFB and proliferation to increase apoptosis
three tablets three times daily

Question 5

Pirfenidone side effects

Answer 5

photosensitivity, gastrointestinal upset and LFT abnormalities

Question 6

Nintdanib

Answer 6

IPD treatment
licensed for cancer treatment
one tablet twice daily
works as an inhibitor of multiple tyrosine kinase receptors, fibroblast growth factor and vascular endothelial growth factor

Question 7

Nintdanib side effects

Answer 7

mainly diarrhoea, but also LFT abnormalities and small increased risk of bleeding

Question 8

Hypersensitivity pneumonitis

Answer 8

main treatment is exposure avoidance and identification, cause unidentified in up to 60% cases

Question 9

HP treatment strategy

Answer 9

focused on inhibition of the adaptive immune system

Question 10

HP treatments

Answer 10

corticosteroids commonly used as first line

Question 11

Corticosteroids

Answer 11

derived from naturally occurring steroids produced by adrenal glands, decrease function of the lymphocyte and cytokine activity

Question 12

Corticosteroids long term consequences

Answer 12

weight gain, GI tract problems, mental health and diabetes

Question 13

Other immunosuppressants

Answer 13

methotrexate, azathioprine, mycophenylate motel, cyclophosphamide, rituximab

Question 14

Methotraxate

Answer 14

inhibitors folate metabolism, reduces T cell ability to make proteins, can cause pneumonitis

Question 15

Azathioprine

Answer 15

Prodrug converted to 6-mercaptopurine, inhibits purine synthesis and reduces T cell turnover.
Can cause hepatitis and bone marrow suppression

Question 16

Mycophenylate

Answer 16

inhibits purine synthesis and reduces T cell turnover, immunosuppressive and susceptible to viral infection

Question 17

Rituximab

Answer 17

Depletion of CD20-positive B-lymphocytes, thereby inhibiting their differentiation into antibody-producing cells
inhibition of T cell co-stimulation

Question 18

Sarcoidosis treatment

Answer 18

not all patients require treatment, favoured in patients that are symptomatic, active inflammation, progressive organ damage and worsening pulmonary function

Question 19

Sarcoidosis and corticosteroids

Answer 19

first line therapy, initial doses of prolnisone

chances of relapse after therapy 14-74%

Question 20

Sarcoidosis and methotrexate

Answer 20

used as second line treatment in patients that are steroid-refractory, typically begin orally with gradually increasing dosing

Question 21

Lung transplantation

Answer 21

HP may recur in the allograft and impact clinical outcomes, vigilance for ongoing antigen exposure and disease recurrence, overall survival is good

Question 22

Palliative treatment

Answer 22

targeted at mitigating symptoms, opioids for breathlessness and pain, anxiolytics and supplementary oxygen

Question 23

Long term oxygen therapy

Answer 23

15+ hours a day
hypoxia at rest
often delivered by static air concentrator

Question 24

Ambulatory oxygen

Answer 24

enables greater exertion to be undertaken in patients with hypoxia on exercise