Pictured above from left to right: Prof John Isaacs (Consultant Rheumatologist), Dr John Allen (Medical Physics), Dr Neil McKay (SpR Rheumatology), Dr Mark Friswell (Paediatric Rheumatologist), Dr Alice Lorenzi (SpR Rheumatology), Dr Valentino Leone (SpR Paediatric Rheumatology), Dr Fai Ng (Senior Lecturer Rheumatology), Prof Helen Foster (Consultant Paediatric Rheumatologist), Dr Bridget Griffiths (Consultant Rheumatologist), Dr Pam Peterson (Consultant Rheumatologist), Dr Lesley Ottewell (Research Registrar)
The Rheumatology Department at the Freeman Hospital, Newcastle upon Tyne, has a specific interest in the connective tissue diseases (CTDs). We are currently looking after more than 200 patients with scleroderma and many others with Raynaud’s. The CTD service is led by Dr Bridget Griffiths, Dr Pamela Peterson (Consultant Rheumatologists) and Sister Karen Walker (Connective Tissue Nurse Specialist) and is also supported by a research registrar, Dr Lesley Ottewell, and rheumatology specialist registrars, who rotate through the department. As a team, we aim to provide supportive clinical care and education as well as pursuing active research in scleroderma, Raynaud’s and the other CTDs. We collaborate with many other departments in Newcastle including respiratory medicine, cardiology, dermatology, neurology and medical physics, thus allowing a comprehensive assessment of patients with scleroderma. These collaborations have resulted in the development of many research projects.
Patients from Newcastle upon Tyne and the surrounding areas of the North East and North Cumbria are usually seen in the dedicated weekly CTD clinic. This clinic is now run by Dr Bridget Griffiths, Dr Pamela Peterson and Sister Karen Walker and supported by a specialist registrar. Dr Ian Griffiths (Consultant Rheumatologist) retired at the end of December 2008 and was also a key member of the team and the service. This team approach allows patients to be looked after by another consultant who is knowledgeable in their condition, when their own consultant is away and the patient develops an emergency. Once a month, the CTD clinic is held in collaboration with Dr Simon Meggitt, Consultant Dermatologist, from the Royal Victoria Infirmary. Adolescent patients with CTDs are referred to us by Prof Helen Foster and Dr Mark Friswell (Consultant Paediatric Rheumatologists), from the regional paediatric rheumatology service. Decisions are undertaken in the outpatient department regarding future treatments, many of which are administered in the day case unit.
Day Case Unit
The day case unit is a 7 bedded unit, occupying one of the bays on our combined rheumatology and orthopaedic ward. It was opened in October 2004. It has expanded and has now become very busy. Last year we treated over 1,500 patients. Patients receive intravenous (IV) treatments in the day case unit. Many patients with scleroderma and Raynaud’s will receive IV epoprostenol. This dilates the blood vessels so improving the blood supply to the hands and feet. Some patients receive this treatment at the beginning of winter for a few days so that the severity of their Raynaud’s is improved and therefore more tolerable. Other patients receive this treatment to heal ulcers on fingers and toes or reverse severe ischaemia, when the blood supply is very poor and the blue phase of the Raynaud’s does not improve.
Some patients with severe scleroderma receive intravenous cyclophosphamide. This helps to reduce inflammation in the lungs or softens rapidly progressive skin thickening/tightening. We also administer intravenous bisphosphanates (treatments for osteoporosis), anti-TNF drugs and rituximab (treatments for autoimmune diseases such as rheumatoid arthritis) on the day case unit. We participate in clinical trials to test new forms of treatment for diseases like scleroderma and these treatments are also often given on the day case unit. We are currently treating 15 patients with scleroderma with IV cyclophosphamide for lung or skin disease and give approximately 120 epoprostenol infusions per year.
As well as providing treatment, the day case unit is a valuable resource for patient assessment, education and support. The dedicated nursing staff on the unit are knowledgeable and familiar with the issues that our patients face on a day to day basis. The day case unit also facilitates patient friendships, enabling patients to meet others with similar conditions which are often uncommon and this is a source of much support for many of our patients. Patient satisfaction with the day case unit is very high.
Collaborative Patient Assessment
Dr John Allen, clinical scientist, specialises in measurements of the small blood vessels (the microcirculation) which are affected by scleroderma and Raynaud’s. Dr Allen leads a state-of-the-art microvascular facility and provides a diagnostic measurement service for the clinical team. He uses a variety of mainly optical techniques, including thermography and nailfold capillaroscopy, to assess patients with Raynaud’s and positive autoantibody blood tests to see who is at higher risk for the development of scleroderma.
A significant number of patients with scleroderma develop pulmonary hypertension. The Freeman Hospital has a National Pulmonary Arterial Hypertension Service Centre. The service is led by Prof Paul Corris and supported by his consultant colleagues, Drs Jim Lordan, Rob Allcock and Guy MacGowan. Once a month Dr Bridget Griffiths also attends the pulmonary hypertension clinic so that patients with connective tissue disease can receive a combined assessment. These opportunities are very important for patients with conditions such as scleroderma as the disease can affect many different organs (such as the lungs) which are best looked after by specialist doctors in combination with a rheumatologist. Decisions can then be jointly made about treatments, their effects and monitoring of the disease. The patient can receive a ‘one stop’ assessment and so reduce the number of hospital appointments.
Dr Peterson has just started a combined rheumatology pregnancy clinic with Dr Jason Waugh, Consultant Obstetrician, from the Royal Victoria Infirmary. Some patients with connective tissue diseases who become pregnant, need careful monitoring and benefit from the combined input of an experienced rheumatologist and obstetrician, specialising in this area.
Role of the CTD Nurse Specialist
Sister Karen Walker (CTD nurse specialist) is the primary contact for many of our patients once a diagnosis of scleroderma has been made. Karen runs the CTD advice line, which means that patients who develop problems in between clinic appointments are able to seek help as required and therefore have ‘open access’ to the service. Her role includes education about the patient’s medical condition, discussion and counselling regarding treatments (e.g. disease modifying treatments which may be methotrexate, mycophenolate mofetil or cyclophosphamide treatment in patients with scleroderma), and provide support to the patient and family regarding any issues they may have relating to the diagnosis.
She is involved in supporting the CTD clinics as well as running nurse-led clinics. Karen acts as an important link between the outpatient clinic and the day case unit. She established a scleroderma patient support group in 2005 which meets on a three monthly basis. Patients meet informally to discuss issues relevant to scleroderma and can support each other. Karen’s salary is now fully funded by the NHS. She works four days per week. The Raynaud’s & Scleroderma Association kindly helped to support her salary for one day a week for a number of years.
Lesley Ottewell began her period of research just over a year ago. She has established a database of all the patients with scleroderma who are seen at the Freeman Hospital. This has enabled identification of patients who have received specific treatments in order to see how effective these therapies have been,
particularly in the treatment of lung and skin disease. Over the last eight years, we have been treating patients with scleroderma with severe inflammation of the lungs and skin with intravenous cyclophosphamide. We believe that by targeting treatment i.e. altering the frequency and duration of this treatment according to the patient’s response, the outcome i.e. improvement is better.
Work is currently underway to look at the usefulness of ultrasound in the assessment of skin thickness in patients with scleroderma and in patients who have the autoantibodies associated with scleroderma and Raynaud’s but do not as yet have thickening or tightening of the skin (pre-scleroderma). We think that the
accurate measurement of skin thickness that can be obtained by ultrasound may be a useful tool for the assessment of the effectiveness of new drug therapies in clinical trials.
Ongoing projects have involved collaboration with Dr John Allen in the medical physics department. Examples of such projects in scleroderma and Raynaud’s patients include the noninvasive assessment of skin composition using fluorescence techniques and the monitoring of cardiovascular health using
new and award winning multi-site peripheral pulse technology. It is hoped that in the future this research work will be of help in understanding the development of scleroderma, its diagnosis and the monitoring of patients’ responses to therapy.
Patients from Newcastle have also participated in large multicentre studies for scleroderma and Raynaud’s including the SSTEPS study, the QUINs study and the RAPIDS2 study. The SSTEPS study was funded by the Raynaud’s & Scleroderma Association and compared oral iloprost, which opens up the blood vessels, versus placebo (dummy drug). The data is currently being analysed. We were a major contributor to this study. We continue to enter patients into multi-centre clinical trials as well as local ongoing studies. Prof Jaap van Laar, who also has an interest in scleroderma, has recently moved to Newcastle University from Leiden in the Netherlands and we collaborate with him and his team.
Lesley Ottewell, Karen Walker and Bridget Griffiths, Connective Tissue Diseases Service,
Department of Rheumatology,
Freeman Hospital, Newcastle upon Tyne