New guideline on systemic sclerosis discussed

Although rare, systemic sclerosis has a significant impact and requires a personalised approach to optimise outcomes and detect life-threatening complications, a leading expert told the Irish Society for Rheumatology 2024 Autumn Meeting.

Prof Chris Denton, Consultant Rheumatologist at the Royal Free Hospital, London, UK, presented an update on advances in treating the disease and highlighted the newly updated British Society for Rheumatology (BSR) guideline for systemic sclerosis, which he helped develop.

The new guideline provides “a comprehensive, up-to-date” road map for evidence-based, expert management of systemic sclerosis in people of all ages, Prof Denton told the meeting.

Since the previous guideline in 2016, there has been major progress in management of systemic sclerosis, particularly in the areas of interstitial lung disease, pulmonary hypertension, and digital ulcer management, he explained.

Prof Denton stressed the importance of early diagnosis and risk stratification for optimal outcomes in systemic sclerosis.

He pointed out the high risk – depending on the subtype/level of disease and antibody levels – of developing interstitial lung disease, pulmonary hypertension, and cancer in these patients and the need for careful monitoring.

In terms of treatment, the BSR guideline recommends that early diffuse cutaneous systemic sclerosis cases should be considered for immunosuppression with mycophenolate mofetil for skin fibrosis. Alternatively, methotrexate may be used for skin fibrosis. “They are more impactful when given early in the disease.”

Multidisciplinary and multispecialty care should be available for systemic sclerosis patients, as per the guidelines, Prof Denton noted.

Specialised treatments include biological agents such as rituximab or tocilizumab, and autologous haematopoietic stem cell transplant “can work very well” in carefully selected patients.

Clinician alertness is key to swiftly manage complications, such as a scleroderma renal crisis, “which can be a lethal outcome if not identified early,” he stated.

While survival is improving, the non-lethal burden of disease is also increasing. This burden, which includes digital ulcers, gastrointestinal complications, calcinosis, and fatigue, can really impact patients’ quality-of-life, Prof Denton acknowledged.

Looking to the future, he said ongoing research could offer potential further disease insight, and better potential treatment options (eg, CAR T-cell therapy), as well as ”improved patient-centred and evidenced-based management”.

Systemic sclerosis is a severe, complex, multi-system disease, and quite uncommon, so the new BSR guideline is particularly important for those with little experience of the disease, Prof Denton told the Medical Independent.

“As systemic sclerosis is a scarring disease where tissue gets damaged, sometimes once that damage has occurred it can’t be reversed,” he said.

“So, in general, always, the earlier you can intervene, the greater the chance of recovery or repair of the tissue,” he said.