Inherited lupus can begin early—and continue silently
A multicenter study led by researchers at Sultan Qaboos University has found that patients with a rare inherited form of lupus may continue to accumulate organ damage even when conventional measures indicate that their disease activity has declined. According to the research team, this represents the largest longitudinal cohort of DNASE1L3 deficiency reported to date.
A new study reveals that patients with a rare inherited form of lupus may continue to suffer organ damage even when their disease activity appears to improve. The research, conducted by a team from Sultan Qaboos University, examined 57 patients with DNASE1L3 deficiency, a genetic disorder that causes systemic lupus erythematosus and hypocomplementemic urticarial vasculitis syndrome.
Despite a significant decrease in disease activity scores, long-term outcomes such as kidney inflammation, lung involvement and mortality worsened over time. This discrepancy suggests that standard lupus activity scores may not fully capture the ongoing immune-complex activity driven by the genetic deficiency, highlighting the need for continued long-term monitoring of at-risk patients.
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