IV Immunoglobulin With Plasmapheresis Improves Outcomes of Churg-Strauss Syndrome Therapy

Yael Waknine

December 10, 2004

Dec. 10, 2004 — The addition of intravenous immunoglobulin (IVIg) synchronized with plasmapheresis to standard therapy with prednisone and cyclophosphamide may reduce the frequency of relapse and the incidence of adverse effects in patients with Churg-Strauss syndrome (CSS), according to the results of a preliminary study published in the December issue of the Annals of the Rheumatic Diseases.

"Corticosteroids and cytotoxic drugs are the cornerstone of therapy, and permit control of disease activity in the great majority of cases," write M. G. Danieli, MD, from the Hematology and Immunology Clinic at the Polo Didattico Scientifico in Torrette di Ancona, Italy, and colleagues. "However, half of the patients relapse within 2 years of achieving remission and experience substantial drug dependent morbidity."

The investigators evaluated the benefit of adding synchronized cycles of 2 g/kg IVIg with plasmapheresis to the therapeutic regimen of nine of 18 patients with CSS being treated with standard prednisone and cyclophosphamide therapy. Cycles in the test group were repeated monthly for six months, followed by three cycles administered every other month.

All patients received 1mg/kg/day of prednisone for one month, followed by a slow dose tapering. Cyclophosphamide was administered at a dose of 2 mg/kg/day for six months in severe cases. Clinical parameters included evaluations of disease activity (Birmingham vasculitis activity scores [BVAS]) and damage index (modified Rankin scores).

Results at one year showed that the addition of IVIg synchronized with plasmapheresis to standard therapy was associated with significantly higher remission rates (100% vs 44%) compared with standard therapy alone.

At three-year follow-up, patients treated with IVIg and plasmapheresis in addition to standard therapy showed significant improvements in BVAS ( P < .01), global damage ( P < .02), and modified Rankin scores ( P < .04), and significant reductions in daily prednisone maintenance dose ( P < .002) compared with those who received standard therapy alone. Patients in the test group also showed a trend toward a decreased incidence of osteoporosis ( P < .057).

"After initiation of the immunoglobulin infusions, we documented a rapid and sustained recovery," the authors write, noting that the clinical and biochemical improvements observed during the first two infusions persisted through the three-year follow-up. "We thus documented the resolution of the main disease manifestations: systemic symptoms, alveolitis, myocarditis, renal impairment, cutaneous vasculitis, polyserositis, and arthritis."

The sustained remission permitted significantly better recovery of peripheral nerve system dysfunction with a significantly lower corticosteroid dose, the authors write, as confirmed by the lower incidence of steroid-dependent adverse events in the test group.

Limitations of the study include small sample size, lack of randomization, and the retrospective analysis of the control group.

"A randomized controlled trial is warranted to confirm the benefit and safety of IVIg in CSS," the authors conclude.

The authors report no pertinent financial disclosures.

Ann Rheum Dis. 2004;63:1649-1654

Reviewed by Gary D. Vogin, MD

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