Clinical features and management of restrictive lung disease

Question 1

what is the problem at hand with obstructive breathing?

Answer 1

the problem is getting air out

Question 2

what is less common but more interesting restrictive or obstructive lung disease?

Answer 2

restrictive lung disease

Question 3

what is a marker of restriction?

Answer 3

vital capacity, spirometry is key

Question 4

state three interstitial lung diseases

Answer 4

idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis

Question 5

what could result in being a restrictive problem in relation to pleural causes of restrictive lung disease?

Answer 5

pleural fluid

Question 6

what are pleural causes of restrictive lung disease?

Answer 6

pleural effusions
pneumothorax
pleural thickening

Question 7

state skeletal causes of restrictive lung diseases?

Answer 7

kyphoscoliosis
ankylosing spondylitis
thoracoplasty - causation of the lungs to collapse
rib fractures - resists ability to breathe

Question 8

state muscle causes of restrictive lung diseases?

Answer 8

amyotrophic lateral sclerosis

Question 9

state sub-diaphragmatic causes of restrictive lung disease?

Answer 9

obesity

pregnancy - restrictive spirometry

Question 10

what is pulmonary interstitium?

Answer 10

it is a collection of support tissues within the lung that include the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues

Question 11

name a group of diseases that lead to lung damage and ultimately fibrosis with loss of the elasticity of the lungs

Answer 11

interstitial lung diseases

Question 12

what are interstitial lung diseases?

Answer 12

a group of lung diseases affecting the interstitium

Question 13

what is the interstitium?

Answer 13

it is the tissue and space around the air sacs of the lungs

Question 14

what may prolonged interstitial lung disease result in?

Answer 14

pulmonary fibrosis, but this is not always the case

Question 15

what is sarcoidosis?

Answer 15

it is a chronic disease of unknown cause characterised by the enlargement of lymph nodes in many parts of the body and the widespread appearance of granulomas derived from the reticuloendothelial system

Question 16

what is idiopathic pulmonary fibrosis?

Answer 16

it is a type of lung disease that results i scarring of the lungs for an unknown reason,

overtime, the scarring gets worse and it becomes hard to take in a deep breath and the lungs cannot take in enough oxygen

Question 17

what is hypersensitivity pneumonitis?

Answer 17

it is an inflammation of the alveoli within the lungs caused by hypersensitivity to inhaled organic dusts, sufferers are commonly exposed to the dust by their occupation or hobbies

Question 18

who gets sarcoidosis?

Answer 18

adults younger than 40
woman more than men
world-wide

Question 19

how do you investigate sarcoidosis?

Answer 19

history and examination

chest x-ray

Question 20

how should mild sarcoidosis be treated? - this is where the patient presents with normal lung function and few symptoms and no vital organ involvement with the condition

Answer 20

no treatment generally

Question 21

how should erythema nodosum be treated?

Answer 21

NSAIDS

Question 22

how should sarcoidosis with skin lesions, anterior uveitis and cough be treated?

Answer 22

topical steroids

Question 23

what disease is considered to have a worse prognosis than lung cancer?

Answer 23

idiopathic pulmonary fibrosis, you are likely to be dead from this condition in less than 3 years after diagnosis

Question 24

what is the typical presentation of idiopathic pulmonary fibrosis?

Answer 24

chronic breathlessness and cough
typical in 60-70 year olds
commoner in men
infection
clubbing

Question 25

what are the options with idiopathic pulmonary fibrosis?

Answer 25

surgery is an option - transplantation
oral anti-fibrotic are a medical option for example pirfenidone and nintedanib
palliative care

the median survival of patients is 3 years

Question 26

what is hypersensitivity pneumonitis also known as?

Answer 26

allergic alveolitis

extrinsic allergic alveolitis

Question 27

what is hypersensitivity pneumonitis?

Answer 27

it is an inflammation of the alveoli within the lung caused by hypersensitivity to inhaled organic dusts

Question 28

what are restrictive lung diseases?

Answer 28

they are a category of extra pulmonary, pleural or parenchymal respiratory diseases

Question 29

describe restrictive lung diseases

Answer 29

these diseases restrict lung expansion, resulting in a decreased lung volume, an increased work of breathing and inadequate ventilation and/or oxygenation

Question 30

state the investigations of restrictive lung diseases

Answer 30

pulmonary function tests

test FVC and FEV1

Question 31

how is sarcoidosis managed

Answer 31

chloroquine

azathioprine

Question 32

how is hypersensitivity pneumonitis managed?

Answer 32

corticosteroids, these can be used in treatment to suppress your immune system, reducing inflammation in your lungs, they are usually taken as a pill

Question 33

how is idiopathic pulmonary fibrosis managed?

Answer 33

nintedanib and pirfenidone

Question 34

why is nintedanib used to treat idiopathic pulmonary fibrosis?

Answer 34

it slows the decline in lung function in mild to moderate cases of idiopathic pulmonary fibrosis

Question 35

why is pirfenidone used to treat idiopathic pulmonary fibrosis?

Answer 35

it is an anti-fibrotic and anti-inflammatory drug