People with early systemic sclerosis may quickly develop skin sclerosis and digital ulcers.
Systemic sclerosis is a rare but serious disease of the connective tissues, where the tissue grows abnormally and triggers the immune system to attack itself (autoimmunity). This can cause hardening (fibrosis) and swelling of the skin, as well as joint pain, digestive problems and lung disease caused by fibrosis and scarring. It usually starts in people aged 30–50 years of age, and is more common in women than men. Skin sclerosis is a key feature of the disease and is most usually measured by assessing skin thickness in 17 different body parts. Digital ulcers are lesions or sores caused by damage to blood vessels. Both skin sclerosis and digital ulcers have a big impact on people with systemic sclerosis. Raynaud’s phenomenon is a condition that affects the blood supply to certain parts of the body – usually the fingers and toes, and may be the first symptom of systemic sclerosis. However, people may have Raynaud’s phenomenon without ever developing systemic sclerosis.
WHAT DID THE AUTHORS HOPE TO FIND?
There is not a lot of information on the development of skin symptoms in early systemic sclerosis. The authors wanted to find out how fast skin sclerosis and digital ulcers developed in people who developed systemic sclerosis within 1 year of having Raynaud’s phenomenon for the first time. They also wanted to assess the skin in different body areas, and work out what factors might be associated with developing skin sclerosis and digital ulcers.
WHO WAS STUDIED?
The study looked at 695 people with systemic sclerosis and Raynaud’s phenomenon. Everyone was over the age of 18.
HOW WAS THE STUDY CONDUCTED?
The study was based on people included in a prospective database called EUSTAR. Data were collected from when the database opened in June 2004 and February 2014. Patients in a registry are not randomised to receive any particular drug, but instead are simply observed and their data recorded. The authors used this information to look at how skin symptoms changed over time, whether there was skin sclerosis present, and if it was present in the elbows and knees, upper arms, chest, abdomen or thighs. They also looked at how often and how fast people got digital ulcers. Finally, the study looked at the effect of people’s gender, age and autoantibody status on skin sclerosis and digital ulcers.
WHAT WERE THE MAIN FINDINGS OF THE STUDY?
Skin symptoms started quickly after the onset of Raynaud’s phenomenon, and peaked on average at 1 year. There was no difference between people with limited or widespread systemic sclerosis. All people who developed moderate to severe skin sclerosis did so within 6.5 years of the onset of Raynaud’s phenomenon, but a quarter of people developed widespread skin symptoms in the first year, and this got less likely over time. The main factors associated with the fast development of widespread skin sclerosis were the presence of two specific autoantibodies in the patients’ blood called anti-RNA-polymerase-III or anti-topoisomerase autoantibodies. Men were more than twice as likely than women to have skin symptoms. The likelihood of developing digital ulcers kept increasing over the 10-year period of the study. The main factor associated with developing digital ulcers early on was the presence of anti-topoisomerase autoantibodies.
ARE THESE FINDINGS NEW?
Several previous studies have suggested that skin sclerosis and digital ulcers are linked to gender, age and autoantibody status. However, before this study, there was only limited information on how quickly those skin symptoms and digital ulcers develop in the early phases of systemic sclerosis.
WHAT ARE THE LIMITATIONS OF THE STUDY?
The study looked at a very specific group of people with systemic sclerosis who had been recruited into the EUSTAR registry within a year of developing Raynaud’s phenomenon. This selection is a limitation of this study because it means that the people included had high skin symptom scores and more widespread skin disease to start with. Therefore, the results must not be generalised to all people who develop systemic sclerosis more than 1 year after Raynaud’s phenomenon. Additionally, the authors say that the evolution of skin symptoms might be underestimated, as some patients already had skin sclerosis at baseline. But by including in this study only patients with a baseline visit within 1 year after the onset of Raynaud’s phenomenon, they tried to keep this effect low.
WHAT DO THE AUTHORS PLAN ON DOING WITH THIS INFORMATION?
The authors hope that the information in this paper may help to design new clinical trials aimed at preventing disease evolution, as well as those investigating new diagnostic tests and drugs.
WHAT DOES THIS MEAN FOR ME?
If you have systemic sclerosis, these findings will help your doctor to give you more accurate information about your disease and how to look after yourself in the early stages.
EULAR recommendations for management. Available at: http://www.eular.org/recommendations_management.
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Date prepared: July 2016
Summary based on research article published on: 31 July 2015
From: Wirz E, et al. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: a 10-year longitudinal study from the EUSTAR database. Ann Rheum Dis 2016;75:1285–92. doi:10.1136/annrheumdis-2015-207271
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