Internal organ involvement ‘much more’ common in late-onset systemic sclerosis
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Key takeaways:
- Pulmonary arterial hypertension and kidney and heart involvement were more common in SSc cases with onset after age 60, vs. early-onset disease.
- Age at onset may be important in “influencing phenotypic variations” of SSc.
Internal organ, heart and kidney involvement, as well as pulmonary arterial hypertension, are “much more” common in cases of systemic sclerosis that develop after age 60 years, according to data published in Clinical Rheumatology.
“According to literature data, approximately 10% to 20% of SSc cases are classified as late-onset, ie, diagnosed after 60 to 65 years of age,” Ewa Wielosz, MD, PhD, DSc, of the Medical University of Lublin, in Poland, and colleagues wrote. “... Controversies still exist on the associated mortality rate in relation to age in SSc, with some reports evidencing that [late-age onset (LAO)] SSc is associated with poorer prognosis and lower survival rates. On the other hand, according to previous studies, LAO SSc was characterized by a milder course with limited morbidity, minimal skin involvement, and limited organ damage.
“It is also unclear whether the frequency of organ involvement correlates with LAO SSc,” they added.
To compare organ involvement and other outcomes in late-onset vs. early-onset SSc, Wielosz and colleagues analyzed the clinical courses and serological profiles of 157 patients with SSc recruited from the rheumatology department at the Medical University of Lublin. The mean age 58.1 years.
Most of the patients had developed SSc prior to age 60 years (n = 118), defining them as early onset, while 39 patients developed SSc after age 60 years and were deemed late onset. Diffuse cutaneous SSc impacted 69 of the patients, while 88 had limited cutaneous SSc.
Compared with patients with early-onset SSc, those with late-onset SSc demonstrated “notably” greater likelihood of pulmonary arterial hypertension (P = .014), heart involvement (P = .0014) and renal involvement (P = .0002), according to the researchers. Patients with late-onset disease also displayed higher levels of anti-Th/To antibodies (P = .014). Meanwhile, arthralgias were more common among those with early-onset SSc (P = .02). The early-onset group also showed a significantly higher prevalence of anti-RNA polymerase III antibodies (P = .008) and antiPM/Scl antibodies (P = .048).
The “poor prognosis” for late-age onset disease “might be related to comorbidities and an increased risk of neoplasms” among older patients with SSc, according to the researchers. Neoplasms affected 15.4% (n = 6) of the late-age onset group and 11.8% (n = 12) of the early-age onset group.
“It has been shown that the course of systemic sclerosis in the elderly differs significantly from that in younger people,” Wielosz and colleagues wrote. “Particularly noteworthy is the fact that involvement of internal organs, heart, kidneys, and pulmonary arterial hypertension, is much more often observed among patients with LAO SSc. Moreover, these groups also differ in their serological profile.
“This is mostly consistent with the results of the majority of similar studies and confirm that age at the disease onset may be an important factor influencing phenotypic variations in the clinical picture and outcomes of patients with SSc,” they added. “Determining these various characteristics and the awareness of the risk of certain organ manifestations in LAO SSc, particularly pulmonary arterial hypertension, should result in special care for these patients and may help to improve the personalized management of the disease.”
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