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Table 2 Clinical features that should prompt suspicion for monogenic lupus/lupus-like

From: Whole exome sequencing in childhood-onset lupus frequently detects single gene etiologies

Early onset – <  10 years of age
Suspected (e.g. recurrent infections) or proved immunodeficiency
Clinical features out of the typical clinical classification criteria for SLE
Severe, life-threatening or organ-threatening presentation
Aggressive course, rapid deterioration and/or accumulation of organ damage
Poor response to treatment
Familial cases
Consanguinity