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Clinical update · 01 of 06

Fulminant vasculitis in intensive care: one in four died by 90 days

Start vasculitis treatment and involve specialists early in critically ill patients with suspected small-vessel vasculitis.

Design
Retrospective single-centre ICU cohort with matched case-control subgroup, 1999–2022
Population
85 ICU patients with acute ANCA-associated vasculitis or anti-GBM disease
Primary outcome
30- and 90-day mortality; end-stage kidney disease
Effect
Mortality 15% at 30 days and 26% at 90 days; 28% of survivors developed ESKD

This single-centre UK series, published in Rheumatology on 22 September, reviewed 85 intensive care admissions between 1999 and 2022 for acute ANCA-associated vasculitis (82%) or anti-GBM disease (15%). Most (80%) were new diagnoses. Median APACHE II score was 19; 92% needed ventilatory support and 66% haemofiltration.

Mortality was 15% at 30 days and 26% at 90 days. Of 90-day survivors, 28% developed end-stage kidney disease. Cyclophosphamide, rituximab and plasma exchange were associated with better survival, and patients treated after 2008 did better, a change not seen in matched ICU controls. The authors link this to changes in the vasculitis service and treatment protocols.

The practical lesson is that patients with pulmonary haemorrhage or rapidly progressive renal failure need prompt vasculitis treatment and early specialist involvement, even when critically unwell. The treatment associations are observational and may reflect who was well enough to receive them.

  • Check ANCA and anti-GBM urgently in any patient with lung haemorrhage and acute kidney injury.
  • Involve the vasculitis or renal team early in ICU admissions with suspected vasculitis.
  • Do not delay immunosuppression for full confirmation when the picture is typical and the patient is deteriorating.
  • Plan renal follow-up for survivors; more than a quarter needed long-term dialysis.
  • Screen for infection alongside, as both can coexist in critically ill patients.

Why it matters

Outcomes improved over two decades as treatment changed, which suggests specialist protocols can save lives in intensive care.

Don't overread it

A single-centre observational series; treatment associations may reflect selection.

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