Developing a new scoring scheme to assess the severity of lung disease in scleroderma

Disease - Scleroderma

Lead applicant - Professor Athol Wells

Organisation - Imperial College London

Type of grant - Project Grant

Status of grant - Active

Amount of the original award - £187,067.16

Start date - 1 August 2014

Reference - 20719

Public Summary

What are the aims of this research?

Lung disease is the major cause of death in scleroderma. The severity of the lung disease in affected patients can be assessed using advanced imaging and lung function tests, but the results don’t always predict how quickly the disease will progress. It may be possible to improve the accuracy of this disease staging using blood tests to look for molecules linked with the disease process. This research will investigate whether it is possible to combine results from clinical tests and blood sample analysis to identify patients who really need treatment. In addition, the link between the presence of inflammatory molecules released from the lungs and worsening skin disease will be explored.

Why is this research important?

Some patients with scleroderma receive unnecessary treatment and others do not receive enough treatment due to inaccurate tests. Combining test results to provide a more accurate picture of the severity of the disease, would be of great benefit to patients and healthcare professionals.

This project will investigate the potential of combining results from blood tests, lung function assessments and advanced imaging of the lungs of patients with scleroderma to produce an overall score of disease severity in individual patients. Researchers will also take small samples of cells from the lungs of patients with scleroderma and examine them in more detail in order to better understand the role of the lungs in scleroderma.

How will the findings benefit patients?

Any progress in identifying which patients should and should not receive treatment with drugs which carry the risk of side effects will be a big step forward in this disease. In addition, improved understanding of the causes of lung disease in scleroderma has the potential to lead to new treatments for this condition.