Churg-Strauss syndrome is a rare type of systemic vasculitis which occurs almost exclusively in patients with asthma and which is characterized by prominent blood and tissue eosinophilia. The disease has a chronic smoldering course with a permanent need for medium to high corticosteroid doses. Available unselective immunosuppressive agents are often insufficient to reduce corticosteroid doses, to induce complete remission and to protect patients from disease flares which occur in more than 50 % of cases. Interleukin-5 is the most potent cytokine regulating the production of eosinophil granulocytes which are the major effector cells in Churg-Strauss syndrome. Recently, an increased production of interleukin-5 was demonstrated in Churg-Strauss syndrome. Mepolizumab is a monoclonal IgG antibody targeting interleukin-5 and is effective in the treatment of the HES. The hypothesis of this study is, that mepolizumab will induce remission and allow for steroid reduction.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
10
750 mg mepolizumab iv q4wk until week 32
University Hospital Schleswig Holstein, Rheumaklinik Bad Bramstetd
Bad Bramstedt, Schleswig-Holstein, Germany
Primary endpoint is the percentage of patients with Churg-Strauss Syndrome that attain remission
Time frame: 52 weeks
Change in BVAS score
Time frame: 52 weeks
Change in Disease Extent Index score
Time frame: 52 weeks
Permanent End organ damage assessed by the Vasculitis Damage Index
Time frame: 52 weeks
Time to remission
Time frame: 52 weeks
Response, defined as a 50 % reduction of the BVAS score
Time frame: 52 weeks
Time to response
Time frame: 52 weeks
The frequency of relapses
Time frame: 52 weeks
Blood eosinophil count
Time frame: 52 weeks
Frequency of all AEs and SAEs
Time frame: 52 weeks
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