Mortality rates are higher in patients with systemic connective tissue diseases (CTDs) and primary systemic vasculitides (PSVs) compared with healthy controls, according to study results published in Rheumatology. Cause of death was often cardiovascular disease, chronic respiratory disease, and infections.

Researchers conducted a prospective case-controlled study analyzing patient data from the Norwegian systemic CTD and vasculitis registry (NOSVAR) over a period of 15 years. The cohort (n=2140) of incident PSV and CTD cases were matched with controls based on age and sex criteria and were randomly drawn from the registry.  The investigators determined cause of death from institutional records and patient death certificates.

After statistical analysis, researchers found that mortality was increased in patients with CTDs and PSVs compared with controls (13% vs 9%; P <.001). Survival rates, measured 10 years following initial diagnosis, were lowest in patients with limited cutaneous systemic sclerosis (75%), anti-synthetase syndrome (73%), and diffuse cutaneous systemic sclerosis (60%). The most common causes of death in the cohort were cardiovascular disease, neoplasm, chronic respiratory disease, and infections; taken together, these accounted for 79% of deaths in the cohort.

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Primary study limitations included the small sample size and lack of precision for each diagnosis.

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“Mortality was clearly increased in patients with CTDs and PSVs compared with the controls. Our results underline the importance of being alert to early symptoms of serious manifestations in CTDs and PVSs and starting treatment as early as possible,” the researchers wrote.

Further studies are needed to fully understand the causes of premature death in patients with systemic CTDs and PSVs.

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Garen T, Lerang K, Hoffmann-Vold A-M, et al. Mortality and causes of death across the systemic connective tissue diseases and the primary systemic vasculitides [published online October 3, 2018]. Rheumatology. doi:10.1093/rheumatology/key285