Determining Disease Activity Biomarkers in Individuals With Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)

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.

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