Fibrotic Lung Disease (Pulmonary Fibrosis) Flashcards
what is pulmonary fibrosis
rare, chronic, life-threatening disease that manifests over several years and is characterised by the formation of scar tissue within the lungs and progressive dyspnoea
what causes pulmonary fibrosis
damage to the alveolar epithelium; cigarette smoke, metal dust, TB, GORD, diabetes and infections
risk factors for developing fibrotic lungs
age, male sex, family Hx, smoking, diabetes, GORD, infection, dust exposure (occupation such as mining etc)
epidemiology
more common among males
about 20 cases per 100,000
presenting symptoms
dyspnoea (gradual onset but progressive course)
dry cough,
wheezing,
fatigue/ weight loss
signs of IPF on physical examination
clubbing, fine inspiratory crackles
in advanced cases of idiopathic pulmonary fibrosis, what cardiac sign can be seen
right sided heart failure
what investigations to do for idiopathic pulmonary fibrosis
CXR, CT, ANA and rheumatoid factor and pulmonary function tests
what is the gold standard test for pulmonary fibrosis
lung biopsy
ABG results?
PO2 decreases with exercise and PCO2 rises as IPF advances
pulmonary function test results in IPF
Restrictive features (reduced FEV1 and FVC, with preserved or increased FEV1/FVC)
Decreased lung volumes
Decreased lung compliance
Decreased total lung capacity
is IPF restrictive or obstructive
restrictive
how will idiopathic pulmonary fibrosis be treated in the acute setting
with high dose corticosteroids
what advice will be given to patients with pulmonary fibrosis
smoking cessation
treatment of ongoing pulmonary fibrosis
cytotoxic therapy OR PPI therapy
