SELECTED ARTICLES FROM THE RECENT LITERATURE 2004

12/15/04

Intravenous immunoglobulin (Ig) therapy in the Churg-Strauss Syndrome

Summary
Background - Treatment of the Churg-Strauss Syndrome (CSS) has often been unsatisfactory with reliance on long-term treatment with cyclophosphamide (Cycl) and corticosteroids (CS) with potential for adverse effects. Therefore, new approaches appear warranted.

Findings - Danieli et al of the Polo Didattico Scientifico in Italy carried out a clinical treatment trial in 18 adults with CSS. All received "standard" treatment initially (prednisone 1 mg/kg/day for one month, then tapering of the dosage plus Cycl 2 mg/kg/day for 6 months). In 9 of the patients (the P-Ig group) synchronized cycles of plasmapharesis and IV Ig (2g/kg) were carried out monthly for 6 months then every other month over the next 6 months. The other 9 CSS patients served as a control group, receiving prednisone as indicated clinically.

After the first 12 months all patients in the P-Ig group and 4 of the 9 in the control group were in remission. After 36 months, there was a significantly lower clinical disease activity (p<0.01) and daily required maintenance prednisone dosage (p<0.002) in the P-Ig group than in the control group. There tended to be a lower frequency of CSS relapses and less osteoporosis in the P-Ig group.

Reference
Ann Rheum Dis 2004;63:1649-54

Editor's Comments
I have reviewed this report because CSS occurs almost entirely in those with underlying active asthma. As such, CSS represents an unusual but very serious development in chronic asthma. The study described above was not based on a randomized, double blind protocol, so one must closely look for objective as well as subjective manifestations of CSS disease activity. Nevertheless, the results of this preliminary study do look encouraging enough to warrant a large controlled study in the future.

 

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