Delayed eculizumab therapy guided by markedly elevated soluble C5b-9 in a child with dialysis-requiring acute poststreptococcal glomerulonephritis: a case report.
Ke Xu, Wei Bai, Suxia Wang et al.
✦ AI-curated · Sources linked
Case in brief
A 9-year-old girl developed gross haematuria, oliguria and stage 3 acute kidney injury after an illness with vomiting and abdominal pain. Positive anti-streptolysin O, low serum C3 and massive proteinuria supported acute poststreptococcal glomerulonephritis. Despite high-dose methylprednisolone and continuous renal replacement therapy, oliguria and renal impairment persisted two weeks after disease onset, while soluble C5b-9 remained markedly elevated. Eculizumab was then given, followed by rapid improvement in renal function. Biopsy showed focal proliferative glomerulonephritis with acute tubular injury. At six months, renal function and C3 were normal, proteinuria had resolved, and only dipstick haematuria remained.
What made this case unusual
Eculizumab was initiated two weeks after disease onset—later than the 2–4-day window described in prior reports—after persistent renal dysfunction and markedly elevated soluble C5b-9 prompted rescue treatment.
Diagnostic / clinical pearl
In severe APSGN with persistent renal dysfunction, ongoing marked soluble C5b-9 elevation may help identify persistent complement activation and inform consideration of delayed rescue eculizumab.
Why it matters
The case suggests that improvement may still follow eculizumab when treatment is delayed, although a single case cannot establish the optimal timing or efficacy.