Avacopan for ANCA-associated vasculitis with hypoxic pulmonary haemorrhage
In a case series of ANCA vasculitis patients with life-threatening hypoxic pulmonary haemorrhage, a population excluded from the pivotal avacopan trial, off-label avacopan use was followed by resolution of bleeding in all patients and rapid steroid tapering.
Background
Pulmonary haemorrhage with hypoxia in ANCA-associated vasculitis carries high early mortality. Avacopan, an oral C5a receptor antagonist approved for AAV, was not studied in patients with pulmonary haemorrhage requiring invasive ventilation, as they were excluded from the pivotal ADVOCATE trial.
Study design
This retrospective, observational, multicenter case series followed 8 AAV patients with hypoxic pulmonary haemorrhage (four requiring mechanical ventilation) who received avacopan alongside standard induction therapy (rituximab and/or cyclophosphamide, plasma exchange) for a median of 6 months.
Key findings
Pulmonary haemorrhage resolved in all 8 patients after starting avacopan (median 10 days from presentation), including two with a month of prior refractory bleeding. Median prednisolone dose fell to 5 mg/day by 1 month, with three patients discontinuing steroids entirely.
Safety
Two patients had serious infections and two discontinued avacopan (one for rash, one temporarily for neutropenia); all patients survived with no re-hospitalization.
Category
Transplant
Source
Nephrology Dialysis Transplantation
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