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. 2004 Dec;63(12):1649–1654. doi: 10.1136/ard.2003.015453

Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome

M Danieli 1, M Cappelli 1, G Malcangi 1, F Logullo 1, A Salvi 1, G Danieli 1
PMCID: PMC1754837  PMID: 15547090

Abstract

Objective: To study the long term effectiveness of intravenous immunoglobulin and plasmapheresis associated with prednisone and cyclophosphamide in Churg-Strauss syndrome.

Subjects and methods: We studied 18 subjects with new onset Churg-Strauss syndrome. All received the "standard" treatment based on prednisone (1 mg/kg/day for 1 month and then slowly tapered) and cyclophosphamide (2 mg/kg/day for 6 months in severe cases). In nine patients, synchronised cycles with plasmapheresis and intravenous immunoglobulin (2 g/kg) were repeated monthly for 6 months and every other month for a further three cycles. Clinical (disease activity monitored by Birmingham vasculitis activity score (BVAS) and damage index (modified Rankin score)) and functional (C reactive protein, blood eosinophil count, and electromyogram-electoneurogram) parameters were collected during treatment and the 3 year follow up period.

Results: After 12 months, all patients in the treatment group and four (44%) in the control group were in remission. At the end of the 3 year follow up period, we documented significant differences in BVAS (p<0.01), global damage (p<0.02), modified Rankin score (p<0.04), and the daily maintenance prednisone dose (p<0.002) between the two groups. We found a tendency towards lower frequency of relapse and incidence of osteoporosis in the treatment group.

Conclusion: Complete clinical and functional recovery with a long term stable remission and a low incidence of side effects can be achieved by intravenous immunoglobulin associated with plasmapheresis in patients with Churg-Strauss syndrome.

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Figure 1.

Figure 1

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 Box plots show the median values (solid line) plus 95% confidence intervals of serum C reactive protein (CRP, mg/dl) for treatment (white bars) and control (shaded bars) groups. At months 6 and 12 and at last assessment, patients receiving IVIg had a statistically significant difference in the CRP serum levels from those treated with a "standard" prednisone/cyclophosphamide treatment (Mann-Whitney U test). Error bars represent the range.

Figure 2.

Figure 2

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 Box plots show the median values (solid line) plus 95% confidence intervals of Birmingham Vasculitis Activity Score (BVAS) for treatment (white bars) and control (shaded bars) groups. At month 12 and at last assessment, patients receiving combined treatment had a statistically significant difference in the vasculitis activity scores than those treated with a "standard" prednisone/cyclophosphamide treatment (Mann-Whitney U test). Error bars represent the range.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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