Interstitial lung disease

Question 1

what is interstitial lung disease

Answer 1

pulmonary fibrosis
diverse group of conditons characterised by varying degress of inflammation or fibrosis
includes idiopathic interstitial pneumonias, multisystem disorders eg. connective tissue disease, sarcoidosis
includes environmental or lifestyle exposures eg. smoking, asbestos, silicosis, drug induced, radiation induced
genetic eg. familial
and others eg. eosinophilic pneumonia

Question 2

signs on phhysical examination

Answer 2

velcro like crackles on chest auscultation
sqeaks and squawks
clubbing of fingers and toes
skin and musculoskeletal sgns of connective tissue diease eg. raynaud phenomenon, sclerodactyly, skin rash, telangeictasia, skin thickening, arthritis, rthralgias and myalgias

Question 3

pulmonary function testing

Answer 3

restrictive pattern
reduced forced vital capacity, normal or reduced FEV1 and normal or increased FEV1/FVC ratio

Question 4

bloods

Answer 4

FBC with eosinophil count
ANA, ENA
dsDNA
serum calcium and RF

Question 5

chest x-ray

Answer 5

may appear normal in mild ILD
high resolution CT may be required, maay show honeycombing

Question 6

what is idiopathic pulmonnary fibrosis

Answer 6

typically occurs in people over 65
more common in males with a history of smoking
if untreated, progressive deterioration and deth generlly occur 3-5 yers after diagnosis

Question 7

treatment of idiopathic pulmonary fibrosis

Answer 7

smoking cessation
pulmonary rehabilitation
drugs for symptom control such as opoiods and anxiolytics for dyspnoea
long term oxygen therapy
early referral for lung trnsplantation
palliative care