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Inherited Lupus Can Begin Early and Cause Silent Organ Damage
Key Takeaways
- A Sultan Qaboos University-led multicenter study examined DNASE1L3 deficiency, a rare inherited disorder that can cause systemic lupus erythematosus and hypocomplementemic urticarial vasculitis syndrome at an early age.
- The cohort of 57 genetically confirmed patients from 22 families in Oman and the UAE is described as the largest longitudinal group of its kind to date, with a median age at diagnosis of five years and a near-equal male-to-female ratio.
- Cases clustered geographically, with 79% of patients from Al Sharqiyah Governorate in Oman and a second cluster in Al Ain, UAE, carrying a different recurrent variant.
- Even as conventional disease activity scores improved, kidney, lung, bone and other long-term complications became more common, a discordance the authors say supports continued organ monitoring into adulthood.
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DT Editorial Team··via medicalxpress.com