People with digital ulcers early in the course of their systemic sclerosis may be more likely to suffer complications of the disease.

INTRODUCTION
Systemic sclerosis is a 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. People with systemic sclerosis are at increased risk of cardiovascular and heart problems. Systemic sclerosis usually starts in people aged 30–50 years of age, and is more common in women than men. Digital ulcers are breaks in the skin that can be painful and disabling. These often occur on the fingertips or finger joints. Because there is a reduced blood flow in these areas, digital ulcers can be slow to heal and may get infected.

WHAT DID THE AUTHORS HOPE TO FIND?
The authors aimed to find out whether people with systemic sclerosis and a history of digital ulcers were more likely to have worse disease progression, and if they were at increased risk of certain complications.

WHO WAS STUDIED?
The study included 3,196 people with systemic sclerosis defined according to the 1980 American College of Rheumatology (ACR) classification criteria. Of these, around one-third had a history of digital ulcers. All patients had at least 3 years of medical follow-up, or were recorded as having died.

HOW WAS THE STUDY CONDUCTED?
This was an observational cohort study performed in the EUSTAR database. EUSTAR collects medical data on people with systemic sclerosis from across Europe. This means that the authors looked at data on a set of patients, but made no interventions and there was no medicine under investigation. All patient records were studied to work out how each person’s systemic sclerosis had progressed during the follow-up period. A statistical analysis was performed to work out whether having digital ulcers before the time people were first seen in the clinic could predict whether people would have worse disease or be more likely to die.

WHAT WERE THE MAIN FINDINGS OF THE STUDY?
People with systemic sclerosis and a history of digital ulcers had a high risk of developing new digital ulcers. They also had increased pressure in their pulmonary artery, increased cardiovascular worsening of the disease, and even a higher chance of death.

ARE THESE FINDINGS NEW?
Yes. This is the first time that digital ulcers in people with systemic sclerosis have been connected to general disease worsening and decreased survival. In clinical practice doctors may look carefully for symptoms of cardio-pulmonary involvement, such as breathlessness, cough and palpitations, but may pay less attention to digital ulcers, especially if they are not very obvious (for example, if they are covered by crusts or thickened skin).

WHAT ARE THE LIMITATIONS OF THE STUDY?
There are some limitations in the way the study was performed. Because this was a retrospective analysis of data in an existing database, it was not possible to look at other information that may have been interesting, such as peripheral vascular involvement, capillaroscopy data, or whether people had high levels of C-reactive protein (or CRP) in their blood, which is thought to be a marker of disease activity and might predict who will have worse disease. However, due to the large number of people studied over an average period of 5 years, the results of the study are strong.

WHAT DO THE AUTHORS PLAN ON DOING WITH THIS INFORMATION?
The authors are performing a new study in the EUSTAR database. The new study will look at gangrene in patients with systemic sclerosis. No further studies on digital ulcers are planned at present, but there is interest in looking at how treatments that reduce blood pressure (vasoactives) might influence disease outcome in people with systemic sclerosis and a history of digital ulcers.

WHAT DOES THIS MEAN FOR ME?
If you have systemic sclerosis and a history of digital ulcers then you are more at risk of developing new ulcers and cardiovascular problems. Patients with systemic sclerosis should have regular checks to ensure that they have good health, but if you are concerned about your risks you should talk to your doctor. You can also talk to the doctor about treatments that are aimed at preventing new digital ulcers, especially during the cold seasons.

Disclaimer: This is a summary of a scientific article written by a medical professional (“the Original Article”). The Summary is written to assist non medically trained readers to understand general points of the Original Article. It is supplied “as is” without any warranty. You should note that the Original Article (and Summary) may not be fully relevant nor accurate as medical science is constantly changing and errors can occur. It is therefore very important that readers not rely on the content in the Summary and consult their medical professionals for all aspects of their health care and only rely on the Summary if directed to do so by their medical professional. Please view our full Website Terms and Conditions.

Date prepared: April 2016

Summary based on research article published on: 16 February 2015

From: Mihai, C. et al. Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis 2016;75:681–86. doi:10.1136/annrheumdis-2014-205897

Copyright © 2016 BMJ Publishing Group Ltd & European League Against Rheumatism. Medical professionals may print copies for their and their patients and students non commercial use. Other individuals may print a single copy for their personal, non commercial use. For other uses please contact our Rights and Licensing Team.