Systemic sclerosis in lockdown – raising disease awareness in unprecedented times

Professor Chris Denton
Dr Voon Ong
Centre for Rheumatology, Royal Free London NHS Foundation Trust

This is a very special time to highlight scleroderma in all its forms, and increase awareness through the annual World Scleroderma Day which falls on 29 June to coincide with the anniversary of the death of the renowned artist Paul Klee, who died from this disease in Switzerland, on 29 June 1940

The disease undoubtedly shaped his art and legacy and his untimely death robbed the world of one of the greatest creative figures of the 20th century.  Eighty years later in 2020, the year has been dominated by the awful pandemic of COVID-19, and this has challenged health services around the world and highlighted the wonderful resilience and capacity of the NHS in the UK.  

It is important to also consider the specific impact of this coronavirus infection on a complex and severe disease like scleroderma (also called systemic sclerosis) and also to reflect on the way that our service has been affected and how we are now emerging to reinvigorate and develop the Royal Free scleroderma activity in clinical care and research.

Systemic sclerosis is an uncommon disease of the immune system that leads to scarring and damage to the skin, blood vessels and internal organs including the heart, lungs, kidneys and bowel.  Ii is not curable but can be treated with powerful drugs, including those which suppress the immune system and some that are also used as cancer chemotherapy.

First, it is important to highlight the consequences of the pandemic for our patients.  They have mostly been shielding due to the complications of the disease including lung fibrosis and pulmonary hypertension and because they are often taking immunosuppressive medication that could increase susceptibility to infection.  This has had a tremendous impact on all those that are shielding but can be especially challenging in a condition that is already socially isolating due to complications and symptoms such as fatigue or bowel involvement and difficulty with social eating.  In addition, normal family support or carers may have been impacted or been unable to visit at home. The detrimental effects of social stress from shielding on the long-term health of our patients are unclear and this may have important clinical implications for the healthcare services. 

Second, there was real concern early in the pandemic that patients with scleroderma might be particularly severely affected.  Now that we are through the peak of infections and can reflect on our experience, it is clear that for scleroderma, as well as other immune mediated disease COVID-19 did not appear to have more severe disease than the general population and almost all our scleroderma patients that did contract COVID-19 have fortunately now recovered.  This is important for us to know if there are future outbreaks, and because it suggests that scleroderma patients are not necessarily any more likely than the general population to develop COVID-19, or to have a worse outcome.  Indeed, some of the drugs used in scleroderma might even treat COVID-19. These important issues will be addressed in ongoing clinical trials and future research and are in agreement with expert recommendations for rheumatic disease (see http://dx.doi.org/10.1136/annrheumdis-2020-217877).  

Another challenge has been the closing of our clinics. This was in line with all non-emergency services in the NHS.  We are now working to prioritise cases and re-instate tests but it will be incredibly challenging as most departments have large backlogs and greatly reduced capacity due to the needs of social distancing and other COVID-related precautions. Within the Royal Free London Foundation Trust, we are working hard to ensure all infection prevention and control procedures are in place to welcome your future clinical visits with us. 

It was disappointing that our 25th anniversary Scleroderma Family Day had to be postponed from May this year.  This would have fallen at the peak of the pandemic and so just as events such as the Systemic Sclerosis World Congress were also missing in 2020, we have had to delay our celebration until next year.  However, we look forward to welcoming as many patients and families as possible for this celebratory Family Day and will look at ways of streaming the content or having it available on line afterwards for those that cannot join us on the day.

Surprisingly, there have also been a few positive consequences.  Like most hospital groups, the Royal Free London NHS Foundation Trust embraced the challenge of COVID-19 and this has really consolidated and cemented professional relationships  and a real “can do” attitude that will hopefully persist to the benefit of patients and services in the immediate future.  There will be more challenges ahead, but we are well prepared and looking to incorporate new ways of working and delivering care.  For example, it seems likely that a component of virtual telemedicine will be part of the future as it works extremely well for some of our patients that live outside London and have shared care with other centres.  In addition, a more integrated way of working and greater degree of collaboration across services was essential at the height of the pandemic.  Specialist physicians, including many from the scleroderma team, joined our colleagues in acute medicine to care for this new disease and there was benefit to colleagues and to patients in having true multi-disciplinary expertise.

Finally, there are likely to be benefits in understanding and treating complex diseases as well as advancing care for any future coronavirus respiratory disease.  Some of the drugs in routine use or clinical trial for diseases such as scleroderma, including corticosteroids and targeted immune suppressive drugs such as tocilizumab could potentially improve outcomes in COVID-19.  In this way complex long-term disease can help to better understand the disease biology of a short term, severe but often self-limiting viral illness.  We have much to learn but global challenges such as this can also have positive benefits despite the almost incalculable loss incurred to far too many families in UK and around the world.