Churg-Strauss Syndrome (CCS) is a rare systemic necrotizing small vessel vasculitis associated with bronchial asthma, peripheral blood eosinophilia and eosinophilic lung infiltration. Skin changes compatible with vasculitis are present in about 75% of patients. Previous reports suggest that patients with CSS can be treated with anti-IgE (omalizumab) in addition to conventional therapy to achieve asthma control. Here we report the efficacy of a 6-month treatment with omalizumab in a patient with CSS characterized by severe asthma and urticarial vasculitis.
Methods:
A 44 year old Caucasian female with a 5 year history of severe asthma, chronic urticaria and mild eosinophilia (1100/μl) was evaluated for possible CSS. Total serum IgE was 662 KU/l with positive skin prick tests for dust mites. Bronchial asthma was not controlled and FEV1 was 60% despite treatment with budesonide (640 mcg/die) and formoterol (18 mcg/die). Diffuse and confluent urticarial rash occurred in the last 6 months before evaluation and respond neither to prednisone (10 mg/die) and rupatadin (10 mg/die) nor to immunosuppressive agents (cyclosporin 200 mg/die or azathioprin 100 mg/die). The patient was treated, as add-on therapy, with omalizumab (300 mg s.c. every 2 weeks) accordingly to total IgE and weight parameters reported in the drug information leaflet.
Results:
After 6 months of treatment the patient reported a significant improvement in asthma control with 50% reduction of nocturnal awakenings and asthma exacerbations and a major FEV1 improvement (101% at 16 weeks and 103% at 24 weeks). Eosinophil count was reduced to 600/μl. A 75% reduction of oral prednisone was registered after 8 weeks of treatment. Importantly, urticarial lesions disappeared after the first injection of omalizumab. Omalizumab injections were well tolerated and no adverse event was recorded.
Conclusions:
This case suggests that omalizumab can be beneficial and safe in patients affected by CSS with severe asthma and urticarial vasculitis. In addition to its effect on serum IgE, efficacy of omalizumab in CSS may be related to an inhibitory effect on blood eosinophilia.