Thursday, 13 April 2017

UK Guidelines for the Treatment and Management of Scleroderma, Raynaud's






In June 2016, Scleroderma Awareness Month  the first national UK guidelines for the treatment of scleroderma (systemic sclerosis) were published in the international journal ‘Rheumatology’, as well as, on the British Society for Rheumatology (BSR) website.
See more here
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.’
Dr Peter Lanyon, Consultant Rheumatologist, has now completed his first year of the 3 year Presidency role of the BSR. Dr Lanyon 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 that the rare disease, non genetic, rheumatological conditions (this includes scleroderma, vasculitis, myositis etc) have been outlined specifically, for areas of improvement, as by the UK Government’s commitment to such by 2020. To read more about this click here
In my view, Dr Lanyon is another unsung hero and blessing to the global scleroderma community, the rare disease community and the musculoskeletal community. I have the pleasure of being a member of the NHS Clinical Reference Group (CRG) for Specialised Rheumatology, which Dr Lanyon chaired prior to taking on his BSR Presidency role. 
I witnessed at first hand, the selfless dedication and extra miles which Dr Lanyon demonstrated in order to improve the day to day reality 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 to take place at the ICC Birmingham 25-27 April 2017. To learn more click here

To watch my presentation ‘Systemic Sclerosis – A Patient’s Perspective’ which I gave at the 2015 BSR annual conference click here

Professor Chris Denton, Royal Free Hospital and UCL Division of Medicine, Chair of the BSR/BHPR Scleroderma Guideline development working group, (another unsung hero and blessing to the global scleroderma community) provides 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 summarised 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 [3].
This is not the end of the process since the guideline 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 [4].

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 centre followed up with management within the framework of a multidisciplinary team.

To read my article about the importance of specialist scleroderma expert centres 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, ASCT.
I am particularly honoured 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:
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
Author Affiliations
  • 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, these 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 who, due to their size and economic climate, are not able 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 have been made available, as well as, identification of the causative factors of scleroderma have been discovered.

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 with this.
   
This article is an update to that published by Scleroderma News in my Column

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April 2017.

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