Back Row from left to right; Kevin Howell, Clinical Scientist, Dr Emma Derrett-Smith, Clinical Research Fellow, Dr Voon Ong, Consultant Rheumatologist, Professor Chris Denton, Consultant Rheumatologist, Dr Svetlana Nityanova, Clinical Research Fellow, Dr Carmen Fonseca, Clinical Research Fellow. Middle Row from Left to right; Dr Revadee Dejthevaporn, Clinical Research Fellow, Dr Gyatari Mittal, Locum Rheumatology Consultant, Dr Anupama Nandagudi, Specialist Registrar in Rheumatology, Dr Niamh Quillinan, Clinical Research Fellow Front Row from left to right; Sister Louise Davis, Clinical Nurse Specialist Connective Tissue Disease, Tanaka Ngcozana, Clinical Nurse Specialist Connective Tissue Disease, Sandra Zimba, Research Nurse The Royal Free Hospital is a major centre for scleroderma and related diseases and cares for more than 1200 cases of systemic sclerosis. This often involves shared care with other units and provides a strong combination of specialist expertise complemented by more local options for providing treatment and performing investigations. As well as specialising in connective tissue disease, our unit also sees the full range of rheumatic diseases in the UK. Our hospital is also a designated national specialist hospital for Pulmonary Arterial Hypertension (PAH). Support from the RSA has underpinned much of our clinical and research work over the past 20 years. In addition the department provides care to local patients for the full range of musculoskeletal/ rheumatic diseases.
Scleroderma Clinic
We hold a dedicated weekly Scleroderma Clinic, which is led by Professor Denton, Dr Brough, Dr Ong, Sister Louise Davis and several Research Fellow Registrars who are working on scleroderma-related projects. Once a month there is a combined scleroderma and dermatology clinic with Dr Orteu here at the Royal Free. Further afield, we have Outreach Clinics at 19 hospitals throughout the UK in places as varied as Sussex, Essex, Wales, Bristol, Portsmouth and Coventry. We have close links with other physicians and surgeons in the hospital who have an interest in scleroderma patients, as the condition can affect many other organs of the body. There is also a longstanding link with Professor Athol Wells and his colleagues at the Royal Brompton Hospital that centres on research and clinical management of cases of scleroderma associated lung fibrosis.
Raynaud’s Clinic
Whereas scleroderma is rare, Raynaud's phenomenon is very common and the large number of cases referred to the unit led us to establish a dedicated clinic for this condition. There is now a Raynaud's Clinic once a week which was established by Sister Helen Wilson and is now run by Sister Louise Davis supported by Dr Nihtyanova and our scientist Kevin Howell. This is a clinic where patients suffering from Raynaud’s are managed and followed up appropriately, and provides an invaluable service to cases of Raynaud's, as it can help to predict whether patients are likely to develop scleroderma or a related condition in the future.
The Connective Tissue Disease Specialist Nurses are involved in supporting patients through education services locally and nationally. This is helpful for patients especially those newly diagnosed, as we are there to provide information and advice and counsel and educate them according to their individual needs. There is also a nurse led advice helpline to support patients in the community, which reassures our patients to know that we are only a phone call away if they need assistance, advice or information (020 7830 2326).
Pulmonary Arterial Hypertension (PAH)
About 1 in 10 people with scleroderma can go on to develop pulmonary arterial hypertension “high blood pressure in the pulmonary artery”. It is thought that this is because scleroderma thickens and stiffens the walls of the pulmonary artery so that it cannot open up easily to relieve the pressure inside it. The pressure inside the pulmonary artery is therefore higher than it should be, resulting in the extra strain on the heart.
Although PAH occurs as a result of a person's scleroderma, it often does not start to develop until many years after the first symptoms of scleroderma appear. Both scleroderma and PAH are very rare conditions. To ensure access to specialist treatments it is important that patients are managed by a designated centre. There are eight designated PAH Centres in the UK based in Newcastle upon Tyne, Glasgow, Sheffield, Cambridge and in 4 London hospitals.
Most patients with scleroderma will undergo regular testing, e.g. annual Echocardiogram, ECG and pulmonary function test, to try and identify early on if PAH or any other problems are developing. We work alongside the scleroderma team to offer a range of options for detecting and treating pulmonary hypertension, including clinical trials. Our aim is to provide co- ordinated patient care with the scleroderma team.
The Pulmonary Hypertension team includes our lead consultant, Dr Coghlan, a specialist registrar and three nurse specialists. As well as regular clinics and out-reach services with the medical and nursing team, we offer an advice line service and a regular telephone clinic for those patients who live a distance from the Royal Free. Treatment options have expanded in the last few years resulting in a much improved outlook for those with scleroderma associated PAH. Trials into new treatments are ongoing constantly and will hopefully produce even more effective treatments.
Thermography
The Royal Free acquired its latest model thermal imager in April 2008: this has now been producing high-quality thermograms for the assessment of Raynaud's and localised scleroderma for more than a year. 2009 is a busy year for Kevin Howell as he is registered for a PhD at the University of Glamorgan (on the topic of thermography, of course!) and hopes to submit his thesis for examination in October.
Two research articles involving thermography are in preparation. Both of these are about the assessment of localised scleroderma, and the first will appear later this year in the journal “Skin Research and Technology.” We also published guidelines for hospitals on the purchase and maintenance of thermal cameras in “Thermology International” in January, and we have an article about laser imaging awaiting publication in the same journal.
After fulfilling his travelling commitments as a tennis umpire at Wimbledon and the US Open this summer, Kevin is off to Germany in September to give two short presentations at the European Congress of Medical Thermology.
Drugs Monitoring Clinic
Although there have been major advances in the treatment of rheumatic diseases over recent years, the use of powerful drugs makes it essential to check bloods regularly for potential side-effects. This is especially relevant to patients with major connective tissue disease or severe arthritis. We have a drug monitoring clinic for patients on DMARDS (Disease Modifying Anti rheumatic Drugs) such as Methotrexate, Azathioprine. Mycophenolate and others. The clinic also caters for patients on newer biological agents such as anti-TNF and rituximab. During these visits patient’s medications are reviewed to ensure that they are taking them as prescribed with no or fewer side effects. It allows patients to discuss their concerns regarding their condition and ensures they are making informed choices about their medication.
Planned Investigation and Treatment Unit (PITU)
PITU is a 20 bedded medical ward with 5 infusion chairs. Development of this facility supported by the dedicated staff that run it, represents a major achievement in delivering high quality care for scleroderma cases at the Royal Free. This unit supports a large number of medical specialities such as Rheumatology, Cardiology and others. All our patient bookings are managed by 2 ward administrators who are responsible for all our bed bookings. Patients are able to contact them Monday-Friday 0900- 1700hrs with regard to their booking and treatments and are able to speak to nursing staff and the Rheumatology team if required. The ward is open Monday- Thursday 24hrs and then closed on a Friday at 2000hrs. Patients come in from home and the majority are independent and self caring. Length of stay will vary according to why they are admitted and it can be from a few hours to 5 continuous.
We arrange investigations, infusions or procedures of which will all be assessed by our Rheumatology team. We are closely connected to the Clinical Nurses Specialists in order to maintain a high level of knowledge and education through their teaching and training on the ward.
Approximately 8-10 scleroderma patients a week come to us for routine iloprost infusions and whilst an inpatient we routinely have cardiac echos, lung function, bloods and renal function assessed. Some patients can go home after a 6 hour infusion; some stay in hospital for the whole duration. All nursing staff on the ward are experienced nurses in Rheumatology specialist diseases and are competent in giving and managing iloprost infusions.
Clinical Trials
We have always placed a very high priority on clinical trials in scleroderma, Raynaud's and PAH. Trials are essential to confirm the effectiveness and safety of new treatments. We have been developing our clinical trials unit over the years as our clinical trials portfolio continues to grow.
The unit participates in national and international clinical trials, single and multi-centre studies lead by Professor Denton and Dr. Coghlan. In the future we are also expecting to do more joint working with Professor Abraham, Head of our Research laboratory. This is because translational research will be more common as attempts are made to reduce the time patients have to wait before new treatments become available. The unit carries out both scleroderma and PAH clinical trials, which we believe are important in helping us to understand scleroderma and evaluate treatments that can improve the quality of life for patients and perhaps ultimately, have a cure for scleroderma and its major complications.
Some of the scleroderma clinical trials that are ongoing or recently completed include: The Gleevec study, Distol study, Detect, P144 and others. We are also eagerly awaiting the results from the recently concluded SSTEP study which has been funded by the RSA. Finally we are also grateful to our patients who continue to volunteer to take part in our clinical trials. You can ring us on 0207 317 7544 for more information.
Translational Research
The RSA also support a wide programme of important and cutting edge translation research into Raynaud's and scleroderma in the Rheumatology Department. Our research aim is to advance the understanding of the disease processes and to assist in the development of prevention and staging strategies in order for improved management and treatment for patients. Broadly, our RSA funded
research looks into the causes of Raynaud's and scleroderma and the way in which we can control disease progression, find useful biomarkers to help in deciding whether the disease is worsening or responding to therapy and ultimately searching for a cure. The projects are often quite technical and usually focus on one or two factors or disease areas of study. The broad research programmes
supported by the RSA will provide a major advance in our understanding of disease mechanism(s) and cover:
• Investigating how genes are controlled in scleroderma and identifying the genes linked to disease in patients with lung involvement.
• Examining the role that key growth factors and cytokines play in disease progression and severity.
• Understating the role of the epithelium skin disease.
The outcomes of these studies will form the basis from which we can identify the most appropriate aspect or area of the disease process, to test the suitability of new compounds and agents in drug development of new therapies that effectively target the disease process.
Summary
In summary, the scleroderma and pulmonary hypertension teams at the Royal Free are working hard to build upon the strong foundations that were built up over the past two decades after Dame Professor Carol Black moved her unit from the West Middlesex Hospital. With invaluable support from the RSA and other medical charities, we have developed a strong programme of research and clinical care that will hopefully continue to serve Raynaud's and scleroderma patients for many years to come.
The following members of the Royal Free team contributed to this article:
Professor Chris Denton, Professor David Abraham, Rachel Ochiel, Jo Hoyland, Kevin Howell, Nike Akinsulire, Sally Reddecliffe, Louise Davis and Tanaka Ngcozana.
Connective Tissue Diseases and Rheumatology Department, Royal Free Hospital, London.
