Idiopathic Pulmonary Fibrosis - pathophysiology, signs and symptoms, investigation and treatment

Idiopathic pulmonary fibrosis is a chronic progressive fibrotic interstitial lung disease of unknown cause that primarily occurs in older adults. Doctors suspect interstitial lung disease when an adult presents with unexplained exertional dyspnoea, chronic dry cough or inspiratory velcro-like crackles on examination. Fibrosis is typically in the lower lobes of the lung.

Clubbing , low oxygen saturation and dyspnoea is the hallmark of idiopathic pulmonary fibrosis. High resolution CT chest is cold standard for diagnosis. Here you see the pathological pattern of usual interstitial pneumonia: low base predominance, traction bronchiectasis, honeycombing and reticular changes without significance ground glass changes.