Demographics: 50y, M>F,

Aetiology

Idiopathic: irritant / immune / genetic

Clinical Features

• dyspnoea, fatigue, dry cough, Wt loss*, Cor pulmonale.
• Dry “Velcro” - inspiratory crackles.

Pathophysiology

• Excess TGF-β & Fibroblasts within lungs

• Patchy, progressive bilateral, interstitial Pneumonia/ Fibrosis + type 2 pneumocyte hyperplasia.

  

Investigations

• Xray: Bilateral, lower lobe reticulo-nodular shadowing.

• CT/MRI: Peripheral honeycombing.

  

• Restrictive PFT, Reduced TLC.

Morphology