Vasculitis & polymyalgia rheumatica

Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study

Ann Rheum DisPublished Retrospective cohort study

Authors' conclusion

These results suggest that dupilumab may be effective in treating patients with EGPA-related ENT manifestations. However, EGPA flares occurred in one-third of patients and were preceded by eosinophilia in 88%, suggesting that caution is required.

Study context

Population
Patients with relapsing and/or refractory eosinophilic granulomatosis with polyangiitis (EGPA) treated with off-label dupilumab, Europe
Participants
51 patients
Outcome
Response (complete: Birmingham Vasculitis Activity Score (BVAS)=0 and prednisone ≤4 mg/day; partial: BVAS=0 and prednisone >4 mg/day)
Follow-up
Median 13.1 months
Methodological detailsobservational, multicentre, off-label use, Primary indication for dupilumab: disabling ear, nose and throat (ENT) symptoms in 92%
  • Observational
  • Multicentre
  • Off-label use
  • Primary indication for dupilumab: disabling ENT symptoms in 92%

Main finding

As reported in the abstract

Complete response

21

41%

Partial response

12

24%

Observational data: associations, not effects.

Further findings

OutcomeValues
EGPA relapse while on dupilumabDupilumab 31%
EGPA relapse while on dupilumab — Relapse associated with blood eosinophilia
14/16 (88%)

EGPA relapse while on dupilumab

Median eosinophil count at dupilumab start significantly lower in relapsers than non-relapsers, as was median time between stopping anti-IL-5/IL-5R and switching to dupilumab

Safety

Adverse events · median follow-up of 13.1 months

Serious adverse events (AEs)
2
Patients reporting AEs: 18 (35%)

Eosinophilia (eosinophil count >500/mm³)

Peak eosinophil count, median (IQR 1268-4501)
2195/mm³
Time to peak, weeks (IQR 4-36)
13
Eosinophilia associated with relapse
41%
Patients with eosinophilia: 34 (67%)

Original publication

Dupilumab for relapsing or refractory sinonasal and/or asthma manifestations in eosinophilic granulomatosis with polyangiitis: a European retrospective study. Ann Rheum Dis. Published 21 September 2023. doi:10.1136/ard-2023-224756

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