Lung Fibrosis

Lung Fibrosis Treatment London

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).

Lung Fibrosis


Investigation is tailored towards the symptoms and signs. Most patients have blood testing, chest x-ray, pulmonary function testing, and high resolution CT thorax.

A lung biopsy may be required if the clinical history and imaging is not clearly suggestive of a specific diagnosis or malignancy cannot otherwise be ruled-out.


If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued.

Many idiopathic and connective tissue-based causes of ILD are treated with corticosteroids,[8] such as prednisolone. Some patients respond to immunosuppressant treatment. Patients with hypoxemia may be given supplemental oxygen.

Hence treatment is different for each disease, as are the outcomes. So diagnosis is important to distinguish the treatment responsive conditions from relatively less responsive ones.

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