Late-age onset systemic sclerosis

Abstract

Systemic sclerosis is a multi-organ connective tissue disease characterised by dysfunction and impaired morphology of the blood vessels with non-specific inflammation and progressive fibrosis. In the majority of cases, the onset is observed between 30-50 years of age; in many cases, however, the diagnosis is established in patients < 20 years of age or > 75 years of age. The course of late-onset systemic sclerosis is markedly different from that in early- onset disease. In late-onset patients, limited systemic sclerosis, pulmonary hypertension, primary heart involvement, and anti-centromere antibodies are more commonly observed. Moreover, the diagnosis of systemic sclerosis in patients > 60 years of age is associated with poor prognosis, higher mortality rates, and an increased risk of neoplasms, as compared to younger patients.