Patients with systemic sclerosis (SSc) with the diffuse cutaneous form of the disease were more likely to develop digital ulcers than those with other subtypes of SSc, researchers said.
Moreover, the ulcers were likely to be associated with other peripheral vascular manifestations of SSc, according to Carles Tolosa-Vilella, MD, PhD, of the Universidad Autonoma de Barcelona in Spain, and colleagues, in an analysis of 1,326 patients enrolled in the Registro De ESCLErodermia (RESCLE) registry.
A history of digital ulcers was least likely in the sine scleroderma SSc subset of patients, the researchers reported in Seminars in Arthritis & Rheumatism.
Most importantly, a history of digital ulcers was a harbinger of a higher mortality risk from both SSc and non-SSc related-causes.
"Digital ulcers are the most common vascular complications of systemic sclerosis," Tolosa-Vilella and colleagues wrote.
"By understanding the SSc features associated with digital ulcers, clinicians may be better able to stratify risk patients and understand the burden of digital ulcers in this disease."
The majority of patients, 60%, in the RESCLE registry had limited cutaneous SSc, while 25% had the diffuse cutaneous form.
Mean follow-up for the entire group was 14 years, and the mean survival from symptom onset was 40 years.
A total of 41.6% of patients in the RESCLE registry reported a history of digital ulcers while 58.4% had no such history.
On multivariate analysis, independent variables associated with a prior or current history of digital ulcers among the entire cohort included younger age when the disease was diagnosed (odds ratio 0.97 (95% CI 0.96-0.98, P<0.001), diffuse cutaneous disease (OR 3.27, 95% CI 2.20-4.85, P<0.001), and Raynaud's phenomenon (OR 4.61, 95% CI 1.78-11.96, P=0.002). Death from any cause also was associated with digital ulcers (OR 1.80, 95% CI 1.20-2.70, P=0.004).
Additional factors that were associated included interstitial lung disease, calcinosis cutis, and acro-osteolysis.
In the subset of patients with diffuse cutaneous SSc, independent associations were seen with digital ulcers for age at diagnosis (OR 0.97, 95% CI 0.95-0.99, P=0.003), telangiectasias (OR 3.43, 95% CI 1.78-6.62, P<0.001), and calcinosis cutis (OR 3.99, 95% CI 1.61-9.89, P=0.003). However, dcSSc patients were less likely than others to exhibit a non-SSc pattern on nailfold capillaroscopy.
Patients in the limited cutaneous SSc subset with digital ulcers were also younger at the time of diagnosis (OR 0.98, 95% CI 0.97-0.99, P<0.001) and had Raynaud's phenomenon (OR 7.18, 95% CI 2.04-25.2, P=0.002). They also more often had calcinosis cutis, interstitial lung disease, and higher all-cause mortality rate than other subtypes.
Death from any cause among patients with the sine scleroderma subtype also was increased (OR 9.67, 95% CI 2.02-46.28, P=0.005).
"Noteworthy [as well], results from our large database confirm that digital ulcer occurrence in SSc patients is a predictor of a poorer survival, with 1.85-fold more deaths from all causes in comparison to patients without history of digital ulcers," the researchers noted.
Mean overall survival for patients with a history of digital ulcers was 57.7% at 30 years.
This was significantly lower than the mortality rate of 73.5% for those who never reported a history of digital ulcers.
Limitations of the study included the usual limitations seen with analyses of registry data such as missing data and loss to follow-up.
Other evidence implicating digital ulcers as a cardinal sign of more extensive disease and worse outcomes in SSc patients included a study in which over one-third of 3,196 SSc patients had a history of digital ulcers on presentation.
On subsequent prospective visits, a history of digital ulcers was predictive of not only active ulcers on follow-up, but it was also associated with worsening of cardiovascular disease and a heightened mortality risk.
And in another study, patients who presented with an active digital ulcer or who reported a history of digital ulcers even in very early SSc were more likely to already have early internal organ involvement than those without any such history.
The authors concluded that digital ulcers may be a "sentinel sign" for early organ involvement in very early SSc.
The study was funded by an unrestricted education grant from Laboratios Actelion. The authors reported no conflicts of interest.
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