medwireNews: Adults who develop systemic sclerosis (SSc) at an older age are at greater risk for rapid disease progression than those with a younger age at onset, research suggests.
“Our findings may have an important impact for recommendations on the number of follow-up visits and additional diagnostics in SSc patients,” say Pia Moinzadeh (University of Cologne, Germany) and team.
The researchers used data from 3281 SSc patients (79.7% female) who were enrolled in the registry of the German Network for Systemic Scleroderma, of whom 54.2% had limited cutaneous (lc)SSc, 34.8% had diffuse cutaneous (dc)SSc, and the remaining 11.0% had overlap SSc.
When the patients were grouped according to age at SSc onset (<40 years, 40–60 years, and >60 years), the researchers found that there was a shift in the distribution of SSc subsets with age.
The 24.5% of patients younger than 40 years at SSc diagnosis had a fairly even distribution of lcSSc and dcSSc, at 43.4% and 41.8%, respectively, while the remaining 14.8% of patients had SSc overlap syndrome. By contrast, the 22.5% of patients older than 60 years at onset were significantly more likely to develop lcSSc, at 65.0%, than dcSSc and SSc overlap syndrome, at 25.7% and 9.3%, respectively.
The authors note that the distribution of SSc subsets “was also reflected by the antibody distribution,” with patients older than 60 years significantly more likely to be positive for anti-centromere antibodies and significantly less likely to be Scl-70 positive than those in the other age groups.
Independent of SSc subset, patients over the age of 60 years developed their first skin changes significantly earlier than those younger than 40 years, despite having significantly lower modified Rodnan Skin Scores, with a mean score of 9.4 versus 10.5 points.
They were also significantly more likely than the early-onset patients to have pulmonary hypertension (19.2 vs 7.5%), heart involvement (14.8 vs 10.5%), dyspnea (57.7 vs 45.8%), hypertension (54.7 vs 19.7%), and proteinuria (23.2 vs 16.7%).
But they were significantly less likely to have esophageal (48.3 vs 55.6%) and musculoskeletal involvement (31.3 vs 38.9%), digital ulcers (34.3 vs 55.4%), and dysphagia (65.3 vs 66.7%).
Over 10 years of follow-up, prognosis was worse for patients older than 60 years at disease onset, as shown by a significantly higher and earlier incidence of organ manifestations compared with the early-onset group of patients and this was seen across all three subsets.
Specifically, older patients were significantly less likely to be free from pulmonary hypertension (61.8 vs 90.5%), lung fibrosis (47.8 vs 56.7%), and heart involvement (67.6% vs 84.3%) at 10 years than their younger counterparts, but musculoskeletal involvement remained less common in the older patients, albeit not significantly, with 57.1% versus 54.5% free from it at this timepoint.
This pattern of organ involvement was seen across all three SSc subsets among the late-onset patients. Patients with dcSSc had the greatest degree of organ manifestation at 10 years. Fewer of these individuals, compared with those with overlap SSc or lcSSc, were free from lung fibrosis (13.9 vs 52.1 and 59.3%, respectively) and heart involvement (55.0 vs 63.1 and 72.5%), but the rate of freedom from pulmonary hypertension was only lower versus the lcSSc group (54.6 vs 53.6 and 65.7%).
Moinzadeh and team conclude in Rheumatology that “an older age at disease onset is an additional risk factor for disease progression in addition to well-known risk factors such as SSc subsets,” and therefore “in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications.”
medwireNews is an independent medical news service provided by Springer Healthcare. © 2020 Springer Healthcare part of the Springer Nature Group