PMID- 7621588 OWN - NLM STAT- MEDLINE DCOM- 19950830 LR - 20190512 IS - 0009-9104 (Print) IS - 1365-2249 (Electronic) IS - 0009-9104 (Linking) VI - 101 IP - 1 DP - 1995 Jul TI - Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin. PG - 2-7 AB - 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), 30 g/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. FAU - Richter, C AU - Richter C AD - Department of Rheumatology, University of Lubeck, Germany. FAU - Schnabel, A AU - Schnabel A FAU - Csernok, E AU - Csernok E FAU - De Groot, K AU - De Groot K FAU - Reinhold-Keller, E AU - Reinhold-Keller E FAU - Gross, W L AU - Gross WL LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Autoantibodies) RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Receptors, Interleukin-2) RN - 9007-41-4 (C-Reactive Protein) RN - EC 3.4.21.- (Serine Endopeptidases) RN - EC 3.4.21.76 (Myeloblastin) SB - IM MH - Adult MH - Antibodies, Antineutrophil Cytoplasmic MH - Autoantibodies/*analysis MH - C-Reactive Protein/analysis MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - Humans MH - Immunoglobulins, Intravenous/administration & dosage/*therapeutic use MH - Male MH - Middle Aged MH - Myeloblastin MH - Receptors, Interleukin-2/immunology MH - Serine Endopeptidases/immunology MH - Vasculitis/*immunology/*therapy PMC - PMC1553313 EDAT- 1995/07/01 00:00 MHDA- 1995/07/01 00:01 PMCR- 1996/07/01 CRDT- 1995/07/01 00:00 PHST- 1995/07/01 00:00 [pubmed] PHST- 1995/07/01 00:01 [medline] PHST- 1995/07/01 00:00 [entrez] PHST- 1996/07/01 00:00 [pmc-release] AID - 10.1111/j.1365-2249.1995.tb02268.x [doi] PST - ppublish SO - Clin Exp Immunol. 1995 Jul;101(1):2-7. doi: 10.1111/j.1365-2249.1995.tb02268.x.