Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)


Rochester, Minn.

Trial status:

Open for Enrollment

Why is this study being done?

EGPA, also known as allergic granulomatosis angiitis, is a systemic vasculitis. EGPA is marked by three distinct symptoms: asthma; eosinophilia, evidenced by an excessive number of eosinophils in the blood and tissues; and vasculitis involving the skin, lungs, nerves, kidneys, and other organs. Nerve involvement may also occur in EGPA, causing pain, tingling, numbness, and muscle wasting in the hands and feet. Because EGPA patients may not show any visible signs of active disease, current methods of monitoring disease progression usually represent a period of extended inflammation and disease activity. Thus, patients may go untreated during a period of undetectable disease when damage might be preventable. This study will use novel scientific methods to identify new biomarkers that can be used to monitor disease activity in EGPA patients. These biomarkers may be used to help direct clinical care for EGPA patients and assist in future drug development. Study visits will occur monthly for the first year, then every 3 months thereafter for the remainder of the study. Blood and urine collection will occur at every visit. A physical exam and medical and medication history will occur every 3 months; also, participants will be asked to complete several questionnaires to assess disease activity, health status, and tobacco, alcohol, and drug use. Participants may have additional study visits if a disease flare or disease-related complications occur during the study.

Who is eligible to participate?

Inclusion Criteria: - Documented evidence of small vessel vasculitis and at least 4 of the following 6 American College of Rheumatology (ACR) criteria for the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss): 1. Asthma 2. Peak peripheral blood eosinophilia of greater than 10% of total white blood cell count 3. Peripheral neuropathy attributable to vasculitis 4. Transient pulmonary infiltrates on chest imaging studies 5. Paranasal sinus abnormalities or nasal polyposis 6. Eosinophilic inflammation on tissue biopsy - Parent or guardian willing to provide informed consent, if applicable Exclusion Criteria: - Granulomatosis with polyangiitis (Wegener's) - Microscopic polyangiitis - Drug-induced vasculitis - Hypereosinophilic syndrome - Sarcoidosis - Infectious forms of vasculitis - Takayasu's arteritis - Giant cell arteritis - Cogan's syndrome - Behcet's disease - Kawasaki disease - Cryoglobulinemic vasculitis - Systemic lupus erythematosus - Rheumatoid arthritis - Mixed connective tissue disease or any overlapping autoimmune syndrome

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