Fibrotic lung disease Flashcards
What causes upper zone fibrosis
C- coals worker pneumoconiosis
H-histiocytosis/hypersensitivity pneumonitis
A- ankylosing spondylitis
R- radiation
T-tuberculosis
S- silicosis/sarcoidosis
What causes fibrosis that predominantly affects the lower zones
Idiopathic pulmonary fibrosis
most connective tissue disorders
drug induced- Nitrofurantoin, amiodarone ,bleomycin, methotrexate
asbestosis
What may someone with fibrotic lung disease present with
progressive exertional dyspnoea
bibasal fine end-inspiratory crepitations
dry cough
clubbing
What investigations need to be carried out
SPIROMETRY - restrictive
FEV1 - normal/decreased
FVC - decreased
FEV1/FVC -increased
CXR
impaired gas exchange - reduced transfer factor -TLCO
What is the gold standard investigation
High resolution CT scan - required to make diagnosis of IPF
Bilateral interstitial shadowing - progresses to honeycombing
How do you manage fibrotic lung disease
First line pulmonary rehabilitation
supplementary oxygen
lung transplant
PIRFENIDONE- can be useful
What is cystic fibrosis
autosomal recessive disorder causing increased viscosity of secretion
due to defect in CFTR gene
How does someone with CF usually present
neonatal period- meconium ileus, sometimes prolonged jaundice
recurrent chest infections
malabsorption
liver disease
What are some other features of CF
short stature
DM
delayed puberty
rectal prolapse - due to bulky stool
nasal polyps
male infertility, female subfertility
What is the sweat test
patients with CF have abnormally high sweat chloride
normal value < 40 , CF indicated by >60
How do you manage CF
regular chest physiotherapy and postural drainage
high calorie diet -high fat intake
patients with CF minimise contact with each other
vitamin supplementation
lung transplantation
Antibiotics in som exarcebations
What other investigations are required for CF
CXR, HRCT and spirometry
sputum microscopy and culture
blood tests
