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

SUMMARY In 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. Clinical and serological evaluation was performed before...

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Bibliographic Details
Published in:Clinical and experimental immunology 1995-07, Vol.101 (1), p.2-7
Main Authors: RICHTER, C., SCHNABEL, A., CSERNOK, E., GROOT, K., REINHOLD‐KELLER, E., GROSS, W. L.
Format: Article
Language:English
Subjects:
Adult
Antibodies, Antineutrophil Cytoplasmic
anti‐idiotypic antibodies
Autoantibodies - analysis
C-Reactive Protein - analysis
Enzyme-Linked Immunosorbent Assay
Female
Humans
Immunoglobulins, Intravenous - administration & dosage
Immunoglobulins, Intravenous - therapeutic use
intravenous immunoglobulin
Male
Middle Aged
Myeloblastin
Receptors, Interleukin-2 - immunology
Serine Endopeptidases - immunology
systemic vasculitis
Vasculitis - immunology
Vasculitis - therapy
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Summary:SUMMARY In 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. Clinical 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.
ISSN:0009-9104
1365-2249
DOI:10.1111/j.1365-2249.1995.tb02268.x