Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients.
OBJECTIVE
To evaluate at 9 months and 24 months the safety and efficacy of intravenous immunoglobulins (IVIGs) administered for 6 months to treat relapses of Wegener's granulomatosis (WG) or microscopic polyangiitis (MPA) occurring either under treatment or during the year following discontinuation of corticosteroids and/or immunosuppressants.


METHODS
Patients received IVIGs (0.5 gm/kg/day for 4 days) as additional therapy administered monthly for 6 months and were assessed every 3-6 months. Corticosteroids could be maintained or reintroduced at the time of relapse; immunosuppressants could be continued but could not be reintroduced. At months 9 (end point) and 24 (followup), the following information was collected: complete or partial remission, relapse as assessed with the Birmingham Vasculitis Activity Score (BVAS) 2005, and tolerance and safety of IVIG therapy.


RESULTS
Twenty-two Caucasian patients (7 men and 15 women) were studied: 19 had WG, and 3 had MPA. Their median age was 53 years (range 19-75 years), and their median duration of systemic vasculitis was 27 months (range 7-109 months). Their median BVAS 2005 score was 11 (range 3-25). At study entry, 21 patients were ANCA positive, and 21 patients were taking steroids and/or immunosuppressants. All patients experiencing relapse were treated with the same drug(s) plus IVIGs. All patients initially responded to IVIG therapy. By month 9, 13 patients had complete remission, 1 had partial remission, 7 had relapse, and 1 had treatment failure. In 8 of the 14 patients who had remission, the response persisted at month 24. Seven patients experienced minor side effects.


CONCLUSIONS
IVIGs induced complete remissions of relapsed ANCA-associated vasculitides in 13 of 22 patients at month 9. Because of the good safety and tolerance profiles of IVIGs, these agents can be included in a therapeutic strategy with other drugs used to treat relapses of WG or MPA.
Martinez
Valérie
V
Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université Paris Descartes, Paris, France.
Cohen
Pascal
P
Pagnoux
Christian
C
Vinzio
Stéphane
S
Mahr
Alfred
A
Mouthon
Luc
L
Sailler
Laurent
L
Delaunay
Claire
C
Sadoun
Alain
A
Guillevin
Loïc
L
D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy* / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012008:Recurrence; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D016756:Immunoglobulins, Intravenous; D011241:Prednisone