Lung inflammation and cystic fibrosis related arthropathy
Research type
Research Study
Full title
Identifying the biochemical signalling pathways involved in lung inflammation and arthropathy in patients with cystic fibrosis
IRAS ID
201570
Contact name
Daniel Peckham
Contact email
Sponsor organisation
University of Leeds
Duration of Study in the UK
3 years, 0 months, 1 days
Research summary
Cystic Fibrosis (CF) is a life-threatening genetic disorder which affects the lungs and digestive system. It occurs in 1:2,500 live births and has a carrier frequency of 1:25. The resulting gene defect causes recurrent lung infections, as well as systemic and pulmonary inflammation which result in progressive lung damage and end stage respiratory failure. In the UK, median predicted survival is 37 with median age of death of 27 years of age.
In the lungs of patients with CF there is an exaggerated inflammatory response which can be seen at birth and has been recognised in CF animal models. This inflammatory response can affect the rest of the body including the joints. Up to 10% of patients report joint disease (arthropathy) which can be very disabling. The mechanism for this inflammatory process is poorly understood, treatment is limited and there are no methods for describing and assessing joint symptoms and impact of quality of life in this population.
Joint diseases depend on the interaction between white blood cells and tissues in inflamed joints. White cells release inflammatory molecules (cytokines); interleukin-1 (IL-1) is one of the key cytokines involved in joint damage and is also involved in inflammation of the lung. Drugs which block IL-1 are effective in diseases of uncontrolled inflammation. Treatment has only been used in one patient with CF and presumed Still’s disease with full resolution of symptoms.
The proposed research is to characterise the symptoms and signs of CF related arthopathy and seeks to identify the biochemical signalling pathways involved in CF related arthropathy (CFA), CF related systemic and airway inflammation. Results will provide the basis for clinical assessment of CF related joint disease and a drug trial of a novel agents for treatment CFA and CF related inflammation.REC name
Yorkshire & The Humber - Bradford Leeds Research Ethics Committee
REC reference
17/YH/0084
Date of REC Opinion
4 May 2017
REC opinion
Further Information Favourable Opinion