2021 Virtual Conference Round-Up

A series of talks took place throughout the day, covering areas of research funded by SRUK, advice on symptom management, and discussions about the future. This article offers a brief overview of each talk as a taster, and in the coming months they will be made available online.

Last Saturday, SRUK hosted our first Virtual Conference. Over 700 people registered, taking part both on the day and catching-up ‘on demand’ later.

This COVID-19-safe version of our usual in-person event hosted talks by leading clinicians and researchers, in addition to a series of stalls in the virtual auditorium where attendees could browse shops, learn more about other SRUK projects, or join in with health and wellbeing workshops.

 A series of talks took place throughout the day, covering areas of research funded by SRUK, advice on symptom management, and discussions about the future. This article offers a brief overview of each talk as a taster, and in the coming months they will be made available online. 

1.The Welcome Talk – Dame Carole Black

Dame Black opened SRUK’s 2021 Virtual Conference with a warm and enthusiastic introduction. Her talk focused on the vast improvement she had seen in the scleroderma research field since the 1970’s when she first witnessed scleroderma as a trainee.  

When Dame Black began her research, there were no specific treatments built for scleroderma – “we borrowed treatments from our rheumatology colleagues, from our respiratory colleagues” and so she was delighted that there were several talks throughout the day focusing on emerging research and treatments specific to scleroderma.

“There is so much to talk about. Compared to when I first got interested in this disease as a young doctor, when I cared for a young woman with scleroderma in the kidneys, she died under my care 3 weeks later. I was determined there should not be a disease that we knew virtually nothing about. I was determined I would do something in scleroderma and make a difference. I can see a flourishing of the dream with so many people making a difference, and SRUK playing a huge part in that.”

2. The Latest Advances – Professor Christopher Denton

    Next up was Professor Denton’s keynote speech, which examined what we have learnt from the latest research, and specifically, what may cause scleroderma and how does it progress.

    Of particular interest was his explanation of the genetic, environmental, and chance factors which combine differently in each individual to put someone at higher risk of developing scleroderma. A definitive cause has not yet been discovered, however ongoing research is revealing more genetic risk factors, several of which were discussed by Professor Denton.

    “Both the environment around you and the environment in your body can be relevant to whether you get the disease.”

    Professor Denton moved on to discuss the latest treatment options, and the hopes that not yet NICE authorised treatments may offer patients for certain aspects of scleroderma such as SSc-ILD. He summarised that the current clinical trials taking place were showing a very positive message, though we are still some distance from a definitive cure.

    3. Can Patient Data Save Lives? – Dr Meghan Jani

      The importance of accurate patient data, and its utility in furthering research, has been particularly highlighted during the COVID-19 pandemic. Dr Jani drew on this comparison during her presentation as she discussed the challenges and solutions of using data to further research and best support patients.

      Her talk included a poll where the audience were asked: ‘Were you asked to shield by the government, and regardless of whether you were asked to or not, did you choose to shield?’

      The varied results demonstrated the confusion in the shielding letter process, which she revealed to be in part the fault of inaccurate or incomplete patient data. Dr Jani then discussed how those challenges are being addressed, including the utilization of AI to understand the unstructured data found in the narrative text of doctor’s notes or outpatient letters.

      Finally, Dr Jani addressed the various sources of information that can be used in research, focusing on the use of social media and social networks such as HealthUnlocked in collecting more holistic patient information which can assist researchers in determining where patients want/need support, and what is a priority to the patient community. In turn, this can help to establishwhat research questions need answering. 

      4. A Practical Guide to Caring for Your Skin, Hands, and Feet – Dr Cate Orteu

        Just before lunch, Dr Orteu, a specialist dermatology consultant, advised the audience on how they could best look after their skin, focusing on the hands and feet.

        Top Tip: Darker skin types often require a greasier moisturiser because of the nature of the epidermis. Very dry skin will respond better to a greasier moisturiser, rather than a lighter lotion.

        She offered a series of do’s and don’ts on how the audience should be looking after their skin, which included:

        • Do moisturise – This maintains the suppleness of the skin
        • Do exercise the hands and feet on a regular basis
        • Do drink hot water / tea, as this can increase skin temperature in 10-30 minutes
        • Do invest in 12% silver socks!
        • Don’t drink coffee / caffeine as this can reduce blood flow to the hands and feet.
        • Don’t smoke as this can reduce blood flow to the hands and feet.

        Her advice was very well received by the audience, promoting a lively Q&A which included questions on the best kind of hand exercises and whether there were other areas of the skin that could be affected by Raynaud’s.

        5. Systemic Sclerosis-associated Interstitial Lung Disease – Dr Elizabetta Renzoni

          Dr Renzoni gave a detailed talk in her breakout room on the causes, risk factors, and assessment process of interstitial lung disease when it is associated with scleroderma. A frequent condition in scleroderma patients, it occurs when the interstitium (a part of the lungs) is thickened by inflammation and scar tissue.

          In healthy lungs, the interstitium is very thin and allows O2and CO2 to pass easily between the air sacs in the lungs, and red blood cells in your blood vessels. When thickened by scleroderma, this passage of gases is reduced, resulting in breathlessness. 

          Due to the asymptomatic nature of the early condition, patients with scleroderma have regular lung function tests and chest high resolution so that it can be identified as early as possible, meaning that treatment can be initiated rapidly. Dr Renzoni discussed how patients can be assessed for whether they have SSc-ILD, and once they have it, how they are monitored for changes in their condition.

          She summarised the clinical trials that areongoing in this area and evaluated the treatment being investigated. Where the data was available Dr Renzoni also talked about the results of the most recent clinical trials. Her closing statement was that: “now there are several targeted therapies available to treat SSc-ILD, clinical trials which look at combinations of those therapies need to begin.”

          6. Does Doctor Know best? –Dr Alain Lescoat and Professor Del Galdo

            Dr Lescoat and Professor Del Galdo’s talk highlighted the importance of building outcome measures that are specific to subtypes of scleroderma (e.g. diffuse and systemic cutaneous SSc) and making sure these measures accurately reflected the subgroups they are built for.

            “An outcome is a symptom or feature that is used in a clinical trial to assess the effectiveness of a treatment. E.g. pulmonary involvement may use cough, or shortness of breath as an outcome. An outcome is the most important aspect of a clinical trial.”

            Dr Lescoat asked the audience what they thought the most bothersome symptom of scleroderma was. He then revealed that this had been studied previously.  

            When asked, patients reported:

            1. Gastro-intestinal
            2. Raynaud’s
            3. Musculoskeletal
            4. Fatigue
            5. Pain

            Clinicians and researchers reported:

            1. Skin involvement
            2. Pulmonary
            3. Quality of life
            4. Digital ulcers
            5. Cardiac involvement

            These factors do not exist in isolation however, they are all interconnected. Dr Lescoat explained that “picking only one of them to create a clinical trial may not be enough, and that a good way of reflecting the overall disease would be to combine symptoms and evaluate them all at the same time using the same tool. This is the idea of the combined response index.”
            Both speakers are working on such a tool for both diffuse and limited scleroderma respectively, by building the CRISS and CRISTAL indexes, which they spoke about in detail.

            7. Face Facts – Dr Elizabeth Price

              Dr Price’s talk had the audience pulling funny faces as she taught us how to measure our mouths, to see whether we had ‘small mouth.’ For those who want to give it a try at home, take your 3 middle fingers, and rotate them so they are vertical, and then see whether you can fit them into your mouth. If you can’t, you may have ‘small mouth.’

              Some top tips for managing this and the other complications scleroderma can bring to the face, such as a dry mouth and tight skin, include:

              • Moisturise
              • Limit sun exposure (as it breaks down collagen in the skin)
              • Don’t smoke (as it has a detrimental effect on collagen)
              • Facial exercising: stretching and improving the opening  - exercised can be found on YouTube. These exercises work only as long as you continue to do them.
              • Limit snacking, as it exposes your teeth to more acid attacks a day than is healthy
              • Consider switching to an electric toothbrush
              • Use a high fluoride toothpaste

              Lastly, Dr Price mentioned the importance of staying positive – it has genuinely been proven to help!

              8. Localised Scleroderma in Children and Young Adults – Dr Clare Pain, Katie Dobson, and Lindsey Clarke

                In a very informative talk, Dr Pain, alongside occupational therapists Ms Dobson and Ms Clarke from Alder Hey Children’s hospital, explored how scleroderma presents in children and young people and the care they can expect to receive in hospitals.

                Following their introduction, a large portion of this breakout session was a Q&A, with a lot of participation from audience members who asked some excellent questions.

                Paediatric scleroderma is incredibly rare, Dr Pain explained, being diagnosed in only 60-80 children a year. Typically, these children will have localised scleroderma, which is diagnosed visually however sometimes a skin biopsy is used for diagnosis instead.

                One audience member asked what the cause of scleroderma in adolescents was, to which Dr Pain responded that whilst there was no definitive cause, there can be genetic links to other autoimmune conditions, and these predisposed children can often have a ‘second hit’, an event that triggers scleroderma such as an illness, or accident which affects the immune system.

                Finally, Ms Dobson and Clarke spoke about the importance of early diagnosis in allowing the child to access psychological support at the earliest opportunity.

                9. Annual Tests – What is Pulmonary Arterial Hypertension in Scleroderma? – Dr Colin Church

                  Dr Church is a consultant physician, who spoke about pulmonary arterial hypertension (PAH) and how it is linked to scleroderma.  PAH affects the heart and the lungs and is when there is unusually high blood pressure in the pulmonary artery, which carries blood from the heart to the lungs.

                  “In pulmonary arterial hypertension, you can think about it like: the same things that’s happening in your fingers in a Raynaud’s attack when the blood flow is reduced, is happening in the lungs. Blood vessels thicken which makes it harder for the blood to flow through, meaning the heart has to work harder.”

                  The related symptoms which can be experienced are varied, and not all people experience the same combination. A recent survey revealed that 48% of people experience symptoms for over a year before being diagnosed, with 40% having to see 4 or more doctors in order for their diagnosis to be confirmed. Speaking from the Scottish Pulmonary Vascular Unit, Dr Church then explained how the process of receiving a diagnosis worked. He talked the audience through a range of diagnostic tests, showing example scans and images from real patients before discussing some of the therapies available to treat different aspects of the condition. 

                  10. Understanding Gastrointestinal Involvement in Scleroderma: The Role of Nutrition – Elizabeth Volkmann

                    Coming to us live from LA, Dr Volkmann’s talk explored the importance of diet in managing any gastrointestinal (GI) symptoms people with scleroderma may experience, and the day-to-day changes that can be made in what to eat, when to eat and how to eat.

                    Over 90% of scleroderma patients will experience some involvement of their GI tract during the course of their disease. Although the causes are not fully understood, Dr Volkmann broke this down into two likely factors:

                    1. Collagen deposition leading to muscle breakdown, blood vessel changes, and problems with the autonomic nervous system which controls digestion.

                    2. An increase abundance of bad bacteria, and decreased abundance of good bacteria.

                      Crammed full of useful tips, Dr Volkmann’s primary advice was to “listen to your body – tune in to how you feel after you eat. Your body will give you the sensations that tell you what foods agree with you and what doesn’t.”

                      11. What Does the Future Hold? – Professor Christopher Denton, Professor Francesco Del Galdo, and Dr Elizabeth Volkmann

                        The final talk of the day was a panel discussion between three leading researchers in scleroderma, which asked the question; ‘What will research into scleroderma look like in 2040? What pioneering treatments are likely to be offered? How will the way care is delivered have changed?’

                        This exciting talk covered a lot of ground and will be discussed in depth in a future research article – no spoilers here!

                        A huge thank you to all the speakers who supported our Virtual Conference with their expertise and time, to all the audience member who engaged in the surveys, quizzes, and Q&As, and lastly to those at SRUK who put in the hours to organise this incredible event! We look forward to showing you all the talks over the next few months, and hope you’ll join us at our 2022 event.