Lung Fibrosis

Pulmonary fibrosis describes a group of diseases which produce interstitial lung damage and ultimately fibrosis. It is a chronic condition characterised by shortness of breath, cough, diffuse infiltrates on chest X-ray and inflammation and/or fibrosis on biopsy.

Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD),[1] refers to that group of lung diseases which affect the interstitium of the lung: that includes alveolar epithelium, pulmonary capillary endothelium, basement membrane, perivascular and perilymphatic tissues. The term ILD is used to distinguish these diseases from obstructive airways diseases.

Many types of ILD will progress to pulmonary fibrosis – a term that represents a spectrum of ‘scarring’ from early stages to established disease that causes architectural distortion of the lung and in the latest stages a pattern known as ‘honeycomb lung’. indeed fibrosis is often a later feature. Idiopathic pulmonary fibrosis refers to fibrotic ILD that cannot be ascribed to a distinct primary cause.

ILD is not a single disease, but encompasses many different pathological processes, some with known causes and others with unknown causes (also referred to as the idiopathic interstitial pneumonias).