Clinical features and management of restrictive lung disease Flashcards
what is the problem at hand with obstructive breathing?
the problem is getting air out
what is less common but more interesting restrictive or obstructive lung disease?
restrictive lung disease
what is a marker of restriction?
vital capacity, spirometry is key
state three interstitial lung diseases
idiopathic pulmonary fibrosis
sarcoidosis
hypersensitivity pneumonitis
what could result in being a restrictive problem in relation to pleural causes of restrictive lung disease?
pleural fluid
what are pleural causes of restrictive lung disease?
pleural effusions
pneumothorax
pleural thickening
state skeletal causes of restrictive lung diseases?
kyphoscoliosis
ankylosing spondylitis
thoracoplasty - causation of the lungs to collapse
rib fractures - resists ability to breathe
state muscle causes of restrictive lung diseases?
amyotrophic lateral sclerosis
state sub-diaphragmatic causes of restrictive lung disease?
obesity
pregnancy - restrictive spirometry
what is pulmonary interstitium?
it is a collection of support tissues within the lung that include the alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues
name a group of diseases that lead to lung damage and ultimately fibrosis with loss of the elasticity of the lungs
interstitial lung diseases
what are interstitial lung diseases?
a group of lung diseases affecting the interstitium
what is the interstitium?
it is the tissue and space around the air sacs of the lungs
what may prolonged interstitial lung disease result in?
pulmonary fibrosis, but this is not always the case
what is sarcoidosis?
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
what is idiopathic pulmonary fibrosis?
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
what is hypersensitivity pneumonitis?
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
who gets sarcoidosis?
adults younger than 40
woman more than men
world-wide
how do you investigate sarcoidosis?
history and examination
chest x-ray
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
no treatment generally
how should erythema nodosum be treated?
NSAIDS
how should sarcoidosis with skin lesions, anterior uveitis and cough be treated?
topical steroids
what disease is considered to have a worse prognosis than lung cancer?
idiopathic pulmonary fibrosis, you are likely to be dead from this condition in less than 3 years after diagnosis
what is the typical presentation of idiopathic pulmonary fibrosis?
chronic breathlessness and cough typical in 60-70 year olds commoner in men infection clubbing
what are the options with idiopathic pulmonary fibrosis?
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
what is hypersensitivity pneumonitis also known as?
allergic alveolitis
extrinsic allergic alveolitis
what is hypersensitivity pneumonitis?
it is an inflammation of the alveoli within the lung caused by hypersensitivity to inhaled organic dusts
what are restrictive lung diseases?
they are a category of extra pulmonary, pleural or parenchymal respiratory diseases
describe restrictive lung diseases
these diseases restrict lung expansion, resulting in a decreased lung volume, an increased work of breathing and inadequate ventilation and/or oxygenation
state the investigations of restrictive lung diseases
pulmonary function tests
test FVC and FEV1
how is sarcoidosis managed
chloroquine
azathioprine
how is hypersensitivity pneumonitis managed?
corticosteroids, these can be used in treatment to suppress your immune system, reducing inflammation in your lungs, they are usually taken as a pill
how is idiopathic pulmonary fibrosis managed?
nintedanib and pirfenidone
why is nintedanib used to treat idiopathic pulmonary fibrosis?
it slows the decline in lung function in mild to moderate cases of idiopathic pulmonary fibrosis
why is pirfenidone used to treat idiopathic pulmonary fibrosis?
it is an anti-fibrotic and anti-inflammatory drug
