Idiopathic pulmonay fibrosis

Question 1

Idiopathic pulmonay fibrosis?

Answer 1

Inflammatory condition of the lung resulting in fibrosis of the alveoli and interstitium. Previously known as cryptogenic fibrosing alveolitis. Occurs in genetically predisposed individuals.

Question 2

define fibrosis?

Answer 2

Pulmonary fibrosis (PF) is a chronic and progressive lung disease where the air sac in the lungs (alveoli) becomes scarred and stiff making it difficult to breathe and get enough oxygen into the bloodstream

Question 3

Idiopathic pulmonay fibrosis aetiology?

Answer 3

Recurrent injury to alveolar epithelial cells results in secretion of cytokines and growth factors This leads to fibroblast activation, recruitment, proliferation, differentiations into myofibroblasts and increased collagen synthesis and deposition

Question 4

Idiopathic pulmonay fibrosis risk factors?

Answer 4

Smoking Occupational exposure to metal or wood Chronic microaspiration Animal and vegetable dusts

Question 5

Idiopathic pulmonay fibrosis epidemiology?

Answer 5

RARE 6/100,000 More common in MALES

Question 6

Idiopathic pulmonay fibrosis symptoms (6)?

Answer 6

Gradual-onset, progressive dyspnoea on exertion, dry irritating cough, NO wheeze, symptoms may be preceded by a viral-type illness Fatigue and weight loss are common.

Question 7

Idiopathic pulmonay fibrosis signs?

Answer 7

Clubbing, bibasal fine late inspiratory crakles, signs of right heart failure in advanced stages of disease.

Question 8

Idiopathic pulmonay fibrosis investigations CXR?

Answer 8

Usually NORMAL at presentation Early disease may show ground glass shadowing Later stage disease shows reticulonodular shadowing, signs of cor pulmonale and eventually honeycombing.

Question 9

Idiopathic pulmonay fibrosis other investigations?

Answer 9

ABG, CT, pulmonary function tests, Bronchoalveolar Lavage (to exclude infection or malignancy)