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The Norwegian connective tissue disease (CTD) and systemic vasculitis (PSV) registry (NOSVAR) studied patient outcomes over a 15 year period and found overall mortality to be higher in the CTD compared to the PSV group.
A prospective registry of CTD and PSV patients enrolled 2140 patients (1534 with CTD, 606 with PSV) between 1999 and 2015. Cases were matched against 15 sex and age matched controls from the Norwegian National Population Registry.
After a mean follow-up time of 9 years, 279 patients (13%) died, compared with 9% of 32086 controls (P < 0.001).
Lowest 10 year survival was seen in diffuse cutaneous systemic sclerosis (dcSSc) at 60%; this was followed by 73% in anti-synthetase syndrome (ASS) and 75% in limited cutaneous SSc patients.
In the CTD and primary systemic vasculitis (PSV) patients, the highest standard mortality ratios (SMRs) included:
- dcSSc SMR = 5.8)
- ASS SMR = 4.1
- Takayasu arteritis SMR = 2.5
- ANCA-associated vasculitis SMR = 1.5)
Overall, the major causes of death were cardiovascular disease (CTD 27%, PSV 28%), neoplasms (CTD 25%, PSV 27%), chronic respiratory disease (CTD 20%, PSV10%) and infections (CTD 9%, PSV 16%).
These data call for strategies to address the excess mortality seen among patients with CTD and PSV.