PE21.4 | Systemic Lupus Erythematosus — Summary & Reflection

KEY TAKEAWAYS

Systemic lupus erythematosus (SLE) is a prototypical type-III hypersensitivity, multi-organ autoimmune disease driven by defective apoptotic clearance, type-I interferon activation, polyclonal B-cell auto-antibody production, and immune complex deposition with complement consumption. Paediatric SLE is more severe than adult-onset disease — higher rates of nephritis (50–80%), CNS lupus, and haematological involvement. SLICC 2012 criteria require ≥4 of 17 criteria with ≥1 clinical AND ≥1 immunological. Key serological markers: ANA (sensitive screen), anti-dsDNA (specific, activity monitor), anti-Sm (specific, not an activity monitor), complement C3/C4 (low = active consumption = flare signal). Lupus nephritis ISN/RPS Classes III and IV require induction with prednisolone + MMF or cyclophosphamide. Hydroxychloroquine is the anchor drug for all patients — the only drug proven to reduce SLE mortality. Monitor: urine protein:creatinine ratio + anti-dsDNA + C3/C4 at every visit. Rising anti-dsDNA + falling complement = pre-empt the flare before the kidneys are damaged.

REFLECT

Reflect on the experience of a 14-year-old girl with SLE who was told for 6 months that her fatigue, hair loss, and joint pain were 'exam stress' and 'adolescent growing pains' before the malar rash and nephrotic proteinuria led to the diagnosis. What clinical cues were present from the beginning that, in retrospect, pointed to SLE? What barriers — physician familiarity, gender bias (dismissing symptoms as emotional), availability of ANA testing — contributed to the delay? How does this case illustrate the importance of considering systemic autoimmune disease in an adolescent girl with unexplained multi-system symptoms? What personal or system-level change would you commit to in order to reduce diagnostic delay for children with SLE in your future practice?