Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin

Abstract

SUMMARYIn this uncontrolled study 15 patients with ANCA-associated systemic vasculitis, who were poor responders to conventional therapy, were treated with single or multiple courses of intravenous immunoglobulin (IVIG), 30g/day over 5 days. Chnical and serological evaluation was performed before and 4 weeks after IVIG. Six of the 15 patients experienced clinically significant benefit from IVIG. Improvement was confined to single organ manifestations (skin, ENT findings), no improvement was seen with conjunctivitis and scleritis, pericarditis or nephritis. No patient experienced complete remission after IVIG. Repeated courses of IVIG at 4-week intervals were no more effective than single courses. In six anti-proteinase 3 (PR3)-positive patients pretreatment sera were incubated with F(ab')2 fragments of the IVIG preparation in vitro to measure the inhibitory effect of IVIG on anti-PR3 activity. An inhibition of anti-PR3 activity by 25-70% was observed; this did not correlate with clinical effects. Approximately 40% of patients benefited from IVIG treatment, though complete remission of disease activity did not occur. Neither clinical characteristics nor the inhibitory effect of the IVIG preparation on serum anti-PR3 activity in vitro predicted clinical response to this treatment modality.

High-dose Intravenous Immunoglobulin Treatment Increases Regulatory T Cells in Patients with Eosinophilic Granulomatosis with Polyangiitis

“…In some patients with EGPA, mononeuritis multiplex, heart failure, or antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis do not respond to the combination therapy of corticosteroids (CS) and cyclophosphamide (CYC) 3,4 . We reported 5 that intravenous immunoglobulin (IVIG) therapy improved severe mononeuritis multiplex or heart failure in patients with EGPA who did not respond to the combination therapy.…”

The place of intravenous immunoglobulin in rheumatic diseases

“…C. Richter и соавт. [53] отметили улучшение кожной и оториноларингологической симпто-матики у 40% больных. Состояние почек (гломерулонеф-рит), органов зрения (склерит, конъюнктивит) и сердца (перикардит) оставалось без динамики.…”

Indications for intra-venous immunoglobulin treatment in rheumatic diseases

“…A ciò si aggiunga il fatto che alcuni dati sperimentali, ottenuti in vitro, hanno documentato la capacità delle immunoglobuline di interferire con il legame degli ANCA al loro specifico antigene attraverso un meccanismo idiotipo-antiidiotipo e di inibire l'attivazione dei neutrofili ANCA-dipendente (49). In due studi in aperto che coinvolgevano rispettivamente 26 e 14 pazienti con persistente vasculite ANCA-associata, è stato dimostrato che il trattamento con IVIG era in grado di determinare una prolungata riduzione dell'attività di malattia rispettivamente nel 75% e 40% dei soggetti (50,51). Allorché le immunoglobuline erano utilizzate come trattamento di prima linea, in assenza di terapia immunosoppressiva specifica in pazienti di nuova diagnosi, quattro pazienti su sei presentavano una prolungata remissione della sintomatologia (52).…”