IVIG Therapy in Granulomatosis with Polyangiitis (GPA)

Introduction to IVIG Therapy

The American College of Rheumatology recommends IVIG as an alternative therapy for patients with active GPA who are unable to receive other immunomodulatory therapy, at a dose of 2 g/kg divided over 5 days 1, 2, 3

IVIG should be considered in patients with GPA who are unable to receive standard immunomodulatory therapies, such as rituximab or cyclophosphamide, according to the American College of Rheumatology 2, 3, 4
IVIG may be considered for refractory disease that has not responded to standard therapies, as suggested by the American College of Rheumatology 5

The total dose of IVIG recommended is 2 g/kg body weight, administered divided over 5 days, as per the Journal of Clinical Oncology guidelines 6
Administering the total dose over 2 days rather than 5 days may increase the risk of treatment-related adverse effects, particularly in pediatric patients, according to Nature Reviews Neurology 7

The recommendation for IVIG in GPA is supported by low-quality evidence, according to the 2021 American College of Rheumatology/Vasculitis Foundation guidelines 2, 3, 8
The optimal duration of therapy and frequency of repeated courses remains undefined in current guidelines, as stated by the American College of Rheumatology 2, 4

IVIG is considered less effective than rituximab or cyclophosphamide for remission induction in most cases, according to the American College of Rheumatology 1, 2
Rituximab is the preferred first-line agent for remission induction in severe GPA when available, as recommended by the American College of Rheumatology and the European League Against Rheumatism 1, 9
For maintenance therapy, rituximab, methotrexate, or azathioprine are preferred over IVIG, according to the American College of Rheumatology and the European League Against Rheumatism 1, 9

IVIG can be safely used during pregnancy when other immunosuppressants are contraindicated, as stated by Nature Reviews Neurology 7