idiopathic pulmonary fibrosis

Interstitial lung disease (ILD) is an umbrella term for lung disease characterised by inflammation and fibrosis of the interstitium. ILD can be idiopathic or secondary to connective tissue disorders, drugs or environmental exposures. Before labelling it as idiopathic we have to rule out secondary causes. ILD is one of the most common extra-articular manifestations of connective tissue diseases (CTDs), causing significant morbidity and mortality.