Pulmonary fibrosis is a progressive incurable scarring disease of the lungs that affects about 30,000 people in the UK. Development of effective therapies will depend on understanding the biology of lung scarring.
Platelet biology and lung fibrosis
We have shown that platelets are hyper-active in patients with idiopathic pulmonary fibrosis (IPF). Platelets contain granules rich in growth factors, with the potential to drive fibrosis when platelets are retained and activated in the lungs. We are studying how platelets contribute to pulmonary fibrosis.
Steroids and pulmonary fibrosis
The PANTHER-IPF clinical trial showed for the first time that steroid-containing treatment regimens unfortunately led to worse outcomes for people with IPF. We wish to harness this unique observation to better understand the roles of corticosteroids in the biology of pulmonary fibrosis.
Molecular imaging of the lungs
We are developing novel imaging techniques in pulmonary fibrosis, both in the lab (in collaboration with Hull University PET imaging centre) and in the clinic.
Symptoms and quality of life
We have an ongoing research program into assessing and treating difficult symptoms in pulmonary fibrosis in conjunction with colleagues in palliative medicine at Hull York Medical School.
About 4,500 people in the UK are diagnosed each year with sarcoidosis, a chronic inflammatory disease that affects the lungs, lymph nodes, eyes, and skin, and sometimes the bones, heart and nervous system. Disabling symptoms such as breathlessness and fatigue lead to impaired quality of life, and loss of work and income, and some patients suffer considerable morbidity and premature death. Pathologically, affected tissues are infiltrated by granulomas composed of macrophages and other immune cells. Current treatments such as steroids temporarily suppress the inflammatory response, but side effects can be personally distressing, disfiguring, and dangerous. Thus, it is important to understand the cellular and molecular drivers of persistent and progressive disease so that patients with sarcoidosis have treatment options that improve or control their disease without causing undesirable side effects.
The monocyte/macrophage in sarcoidosis
Recent evidence has highlighted key roles for tissue macrophages and their precursors, blood monocytes, in driving sarcoidosis pathology. We are studying immune responses in the blood and lung tissue of patients with sarcoidosis to help understand how the disease is initiated and perpetuated. We are particularly interested in how abnormal function of regulatory (inhibitory) receptors on blood monocytes leads to an overactive immune response in sarcoidosis.
Thanks to our funders:
British Lung Foundation
Sir Jules Thorn Charitable Trust
Foundation for Sarcoidosis Research
2016-2019 Ms Emma Welch, PhD (Hull York Medical School). Sir Jules Thorn Research Trust. Mechanisms and consequences of platelet activation in idiopathic pulmonary fibrosis. Lead supervisor
2016-2019 Ms Francesca Longhorne, PhD (Hull York Medical School). University of Hull/HYMS research studentship. Immunological mechanisms in pulmonary fibrosis. Lead supervisor
2013-2016 Mr James Thompson, PhD (Hull York Medical School). University of Hull/Hull York Medical School research studentship. Development of novel PET imaging tracers in pulmonary fibrosis. Lead supervisor
2012-2015 Mr Simon Fraser, PhD (Hull York Medical School/University of Hull), awarded 2017. University of Hull/HYMS research studentship. Immunological studies in sarcoidosis. Lead supervisor
2012-2015 Mr James Williamson, PhD (Hull York Medical School/University of Hull), awarded 2016. University of Hull/Hull York Medical School research studentship. The role of endothelial adhesins in leukocyte adhesion in an experimental model of pharmaceutical agent-induced pulmonary fibrosis. Lead supervisor
2008-2011 Ms Ai-Yen Chin, PhD (University of Hull/Renal Research Foundation/Respiratory Medicine), awarded 2013. Protease activated receptor-4 in lung and renal fibrosis. Lead supervisor
2008-2011 Ms Aikaterina Bazakou, MPhil (University of Hull), awarded 2013. Regulation of Fc receptor function. Lead supervisor2009-2011 Dr Michael Crooks, MD (University of Hull), awarded 2012. Platelet and endothelial function in pulmonary fibrosis. Lead supervisor
2008-2010 Dr Ahmed Fahim, MD (University of Hull), awarded 2011. Pathogenesis of idiopathic pulmonary fibrosis. Lead supervisor2010 Dr Chee Kay Cheung, MSc by research (Hull York Medical School), awarded 2011. PAR4 in renal fibrosis. Lead supervisor.