During Scleroderma Awareness month in June, the first national U.K. guidelines for the treatment of scleroderma (systemic sclerosis) were published in the international journal of Rheumatology, as well as on the British Society for Rheumatology (BSR) website. These guidelines were put together by the BSR and BHPR (British Health Professionals in Rheumatology) “to develop an expert driven evidence based series of recommendations for the management of scleroderma.”
Earlier in the year, the BSR held its annual conference in Glasgow, with Scleroderma News reporting on day 2, thanks to Will Gregory; and day 3, thanks to Prof. Chris Denton.
At the annual conference, the newly elected President, Dr. Peter Lanyon, Consultant Rheumatologist, was installed into his 3-year presidency.
Dr. Lanyon recently set out his plans and vision for an improved musculoskeletal-rheumatological healthcare-patient experience, with an “all hands on deck” approach. (Although, sclerodactyl hands may not be much use, if taking this in a literal sense.) It is very encouraging to see rare disease, non genetic, rheumatological conditions (including scleroderma, vasculitis, myositis and so on) outlined specifically for areas of improvement by 2020. To read Dr. Lanyon’s vision, click here.
In my view, Dr. Lanyon is another unsung hero and a blessing to the global scleroderma community, the rare disease community and the musculoskeletal community. I had the recent pleasure of being a member of the NHS Clinical Reference Group for Specialized Rheumatology, which Dr. Lanyon chaired. I witnessed first hand, the selfless dedication and extra miles he demonstrated in order to improve the day-to-day realties and care for patients — as well as improving the standards of the NHS as a world-class leading provider of healthcare.
The next annual BSR conference is scheduled for 25-27 April 2017, at the ICC Birmingham. To learn more, click here.
Professor Chris Denton, Royal Free Hospital and UCL Division of Medicine, Chair of the BSR/BHPR Scleroderma Guideline development working group, provided the following helpful executive summary to the UK national scleroderma guidelines, and, an insight into the important background to their formulation:
‘These guidelines are important because they summarized the current best practice for treating the major complications of systemic sclerosis and also address the overall approach to disease management in the UK. They have been developed under the auspices of the SAGWG (Standards, Audit and Guidelines Working Group) of BSR that has developed a process that is accredited by NHS evidence. This is important since it means that the guideline is NICE accredited and should therefore be taken very seriously within the NHS as defining the standard of care for patients and access to therapies. Embedded within the guideline are important NHS England policies for the management of digital ulcers and the pathway developed for assessment and delivery of autologous stem cell transplantation for appropriate cases of diffuse systemic sclerosis.
The guideline process involved establishing a development group that included rheumatologist, scleroderma experts, pharmacists, allied healthcare professionals, specialist nurses, primary care representatives and patients. In this way all aspects of the disease and management could be included. A comprehensive literature review of all the evidence supporting treatments for scleroderma was an important starting point and a group of dedicated clinical fellows undertook this work. There was a series of telephone and face to face meetings over 2 years that led to the development of the draft guideline. This was then reviewed by BSR SAGWG and by external referees. Comments were incorporated and the revised guideline was then finalised and submitted for open consultation so that anyone could comment and have input. After this process the final guideline was written, this was submitted for approval of BSR and then for publication in Rheumatology.
This is not the end of the process since the guidelines are reviewed and updated every 5 years according to NHS Evidence protocols. This is a landmark for UK scleroderma patients and an important one at a time of major NHS change and also challenged and competition for resources for rare diseases. It complements the other recommendations being updated such as this of EULAR (European League Against Rheumatism) and the UKSSG (UK Scleroderma Study Group) best practice consensus documents.
The structure of the UK national guideline is divided into 3 sections:
Part A: general approach to SSc management
This section highlights the importance of an early diagnosis, with referral to a specialist scleroderma center followed up with management within the framework of a multidisciplinary team.
To read my blog post about the importance of specialist scleroderma expert centers, click here.
Part B: key therapies and treatment of organ-based disease
This section highlights all of the major complications and potential organ involvement, as well as possible treatment options.
Topics include: Raynaud’s phenomenon, digital ulcers, lung fibrosis, pulmonary arterial hypertension, gut involvement, renal involvement, cardiac manifestations, skin manifestations, calcinosis, musculoskeletal manifestations, and ASCT.
I am particularly honored to have been a member of the medical team who compiled the current NHS guidelines for digital ulcer management in scleroderma. To view these guidelines, click here.
Part C: service organization and delivery within NHS England
Scleroderma (SSc) should be diagnosed promptly, investigated appropriately, and managed within an integrated system of primary, secondary and tertiary level care.
The guidelines are scheduled to be reviewed by the NHS in 2019.
References for more information:
• 3. BSR Guideline executive summary – Rheumatology 2016
• 4. UKSSG Consensus Best Practice Documents available at: http://www.scleroderma-royalfree.org.uk/UKSSG.html
Authors of BSR and BHPR guideline for the treatment of systemic sclerosis
- Christopher P. Denton1, Michael Hughes2, Nataliya Gak1, Josephine Vila3, Maya H. Buch4, Kuntal Chakravarty1, Kim Fligelstone1, Luke L. Gompels5, Bridget Griffiths3, Ariane L. Herrick2, Jay Pang6, Louise Parker7, Anthony Redmond4, Jacob van Laar8, Louise Warburton9, Voon H. Ong1, on behalf of the BSR and BHPR Standards, Guidelines and Audit Working Group
- 1Centre for Rheumatology, Royal Free Hospital, London
- 2Rheumatology Department, Salford Royal NHS Foundation Trust, The University of Manchester, Manchester Academic Health Science Centre, Manchester
- 3Department of Rheumatology, Freeman Hospital, Newcastle upon Tyne
- 4Leeds Institute of Musculoskeletal and Rheumatic Medicine, Chapel Allerton Hospital, Leeds
- 5Rheumatology Department, Musgrove Park Hospital, Taunton,
- 6Pharmacy Department
- 7Centre for Rheumatology, Royal Free Hospital, London, UK
- 8Rheumatology and Immunology, UMC Utrecht, Utrecht, The Netherlands
- 9Primary Care, Telford and Wrekin NHS Trust, Telford, Shropshire, UK
For the scleroderma patient, the guidelines are a quantum leap in progress for scleroderma best practice management and care. These guidelines can be used universally around the world, especially for those countries that because of size and economic climate, are unable to produce such recommendations for their scleroderma patients.
As with all things, change is a constant. I very much hope that by the time of the NHS scheduled review for the guidelines in 2019, more and improved treatments will have been made available, as well as identification of the causative factors of scleroderma.
It is with huge gratitude and thanks to the super-human Prof. Chris Denton for championing the formulation of the best practice management guidelines for the treatment of scleroderma in the UK, along with the numerous expert medics who assisted.
Note: Scleroderma is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this blog article are not those of Scleroderma News and are only intended to spark discussion about issues pertaining to the disease.
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