title: Granulomatosis with polyangiitis and intravenous immunoglobulins: a case series and review of the literature.
abstract: Granulomatosis with polyangiitis, formerly known as Wegener's granulomatosis or disease, is a systemic, necrotizing small-vessel vasculitis, belonging to the group of anti-neutrophil cytoplasm antibody vasculitis. The therapeutic strategy includes, in most cases, corticosteroids associated, at least in severe forms of the disease, with immunosuppressive agents: cyclophosphamide and rituximab to induce remission, methotrexate, azathioprine and mycophenolate mofetil to prevent relapses. Intravenous immunoglobulins represent an alternative adjuvant therapy. We described 5 cases of patients with granulomatosis with polyangiitis treated with monthly high-dose intravenous immunoglobulins (500mg/kg/daily for 3 consecutive days for 9months). No patients experienced adverse reactions, and 4 patients (80%) achieved a complete remission after 9 courses of this therapy, which was maintained also 3months later, although we are unable to determine whether improvement in outcomes was a direct result of the IVIG. We also discussed the beneficial effects of intravenous immunoglobulins in patients suffering from granulomatosis with polyangiitis, reporting the previously published data.
authors: Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy. Electronic address: giacoguidelli@hotmail.com.
authors: Giacomo Maria
authors: GM
authors: Guidelli
authors: Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
authors: Sara
authors: S
authors: Tenti
authors: Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
authors: Nicola Antonio
authors: NA
authors: Pascarelli
authors: Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
authors: Mauro
authors: M
authors: Galeazzi
authors: Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
authors: Antonella
authors: A
authors: Fioravanti
mesh_terms: D000328:Adult; D000368:Aged; D004359:Drug Therapy, Combination; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy* / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012074:Remission Induction
chemical_list: D016756:Immunoglobulins, Intravenous
keywords: ANCA-vasculitis; Granulomatosis with polyangiitis; Intravenous immunoglobulins