United Kingdom Primary Sjogren's Syndrome Registry (UKPSSR)

Primary Sjogren s syndrome (PSS) is a chronic condition that affect up to 3% of adults in the United Kingdom (UK). Although the commonest symptoms of PSS are dry eye, dry mouth, joint pain and profound fatigue, almost any part of the body can be affected. Moreover, PSS is associated with 40 times higher risk of developing lymphoma (a form of cancer). Patients with PSS also have poor quality of life and a significant proportion of PSS patients are unable to work because of their disease. Unfortunately, we know very little about what causes PSS and we have not found any effective treatment. However, several recent developments in PSS research have created a platform for finding new treatment for PSS and improving our understanding of the underlying disease processes of PSS. Firstly, the UK Sjogren?s Interest Group (UKSIG) have developed standardised methods to assess patients with PSS, so that we can test how good a specific treatment is and make comparison with other treatments. Secondly, a number of promising therapies for PSS have been developed in the last few years. Thirdly, emerging new research technologies offer new approaches in PSS research. The aim of this project is to capitalise on these recent developments by creating a database of 500 patients with PSS over a period of 3 years so that new treatments for PSS can be tested more quickly and efficiently and clinical studies can be carried out more effectively. We need so many patients because not all patients are suitable for all studies. We will assess the patients using the standardised methods mentioned earlier and blood samples from these patients will be stored for future research. Patients will be recruited from 24 hospitals across the UK and the assessment will be carried out at the local clinics. This is a UKSIG project, co-ordinated by doctors from Newcastle University, the Freeman Hospital and University Hospital of Birmingham. Many doctors involved in this project have participated in previous multi-centre studies organised by the UKSIG. The proposed database will provide a unique and valuable resource for clinical and academic studies as well as for identifying suitable patients for clinical trials. The database will make it quicker and easier to organise these studies. Furthermore, this project will encourage collaborative research between academia, National Health Service and industry, and will promote the use of standardised assessments in the management of patients with PSS.

Primary Sjogren s syndrome (PSS) is a chronic multi-system disease that affect up to 3% of adults in the United Kingdom. Although sicca symptoms, joint pain and fatigue are the commonest symptoms of PSS, almost any organs can be affected and there is a 40-fold increased risk of developing lymphoma. However, little is known regarding the pathogenesis and effective treatment has not been identified. The recent development of standardised outcome measures by the UK Sjogren‘s Interest Group (UKSIG) and others have generated criteria by which the effectiveness of specific therapy can be judged and compared. Furthermore, emerging biological treatments, notably anti-B cell therapies, have shown promising results in improving fatigue, sicca symptoms and systemic features of PSS. These recent developments have provided the framework for testing new therapies in PSS and performing other clinical studies. The aim of this project is to create a cohort of 500 well-characterised PSS patients in order to facilitate such proof-of-concept clinical trials, observational and longitudinal studies in PSS. 500 patients is required because not all patients are suitable for all studies. PSS patients will be identified from existing databases of the 24 collaborating hospitals. After obtaining written consent, patients will be assessed for severity of the key symptoms, disease activity and organ damage and health-related quality of life. These assessments include patient-completed questionnaires, physical examination, bedside clinical tests, laboratory tests of peripheral blood and urine. All assessments will be completed in a single outpatient visit, and all laboratory tests are readily available in all recruiting hospitals. In addition, we will store serum, DNA and RNA samples from patients for future studies. We will develop a database software specifically for this registry. All clinical data and biological materials will be stored in anonymised-linked format. The proposed registry will provide a unique and valuable resource for clinical and academic studies as well as for identifying suitable patients for clinical trials and other clinical studies. The creation of a well-characterised cohort of PSS patients will significantly reduce the cost of screening patients for these studies and will increase the appeal for sponsorship from industry for these proof-of-concept studies. This project will also foster collaborative research between academia, National Health Service (NHS) and industry, and promote the use of standardised assessment tools in managing patients with PSS. Finally, since this cohort will be the largest of its kind, it will also generate invaluable epidemiological data.