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Churg-Strauss Syndrome (CSS) is a necrotising vasculitis of medium and small sized vessels clinically characterised by asthma, eosinophilia, and systemic vasculitis associated with circulating antibodies to antineutrophil cytoplasmic antigen (ANCA). The standard treatment of this condition comprises corticosteroids and, in severe cases, cytotoxic drugs. However, this treatment protocol is associated with a high relapse rate and significant drug dependent side effects.
In this prospective study 18 patients with new onset CSS were evaluated and divided into two groups. While all patients received the standard treatment of prednisolone (1 mg/kg/day for 1 month and then tapered) and, in severe cases, cyclophosphamide (2 mg/kg/day for 6 months), nine patients in the treatment group received “synchronized therapy” comprising cycles of plasmapheresis and intravenous immunoglobulin (2 g/kg). This was repeated monthly to 6 months, then bi-monthly for another three cycles. All patients were evaluated monthly for 1 year and subsequently followed up for 3 years. At the end of 1 year all nine patients in the treatment group and four in the control group were in remission. At the end of 3 years there was a clinically and statistically significant reduction in disease activity in the treatment group as measured by the Birmingham Vasculitis Activity Score (BVAS) and modified Rankin score. The frequency of relapse was also lower in the treatment group (1/9) compared with the control group (4/9). Finally, the daily maintenance dose of prednisolone required was less in the treatment group, as was the incidence of steroid induced side effects such as osteoporosis.
This study suggests plasmapheresis and intravenous immunoglobulin to be a new yet safe treatment option for CSS as an add-on to the standard treatment protocol. Further studies with larger sample size are needed to confirm the evidence generated by this study.