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

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ClinicalTrials.gov Identifier: NCT00315380

Recruitment Status : Recruiting

First Posted : April 18, 2006

Last Update Posted : April 25, 2018

See Contacts and Locations

Sponsor:

Collaborators:

Office of Rare Diseases (ORD)

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)

Rare Diseases Clinical Research Network

Information provided by (Responsible Party):

Peter Merkel, University of Pennsylvania

Study Description

Brief Summary:

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) is a rare immune system disorder that causes asthma, an excessive number of eosinophils (a type of white blood cell) in the blood, and the inflammation of blood vessels, or vasculitis. In order to properly treat EGPA, it is critical that the level of disease activity can be determined over the course of the disease. The purpose of this study is to determine new biological markers, or biomarkers, that may be used to assess the severity of this disease in people with EGPA.

Condition or disease
Eosinophilic Granulomatosis With Polyangiitis Churg-Strauss Syndrome

Detailed Description:

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.

Study Design


Study Type :	Observational
Estimated Enrollment :	500 participants
Observational Model:	Cohort
Time Perspective:	Prospective
Official Title:	Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)
Study Start Date :	April 2006
Estimated Primary Completion Date :	April 2019
Estimated Study Completion Date :	April 2019

Resource links provided by the National Library of Medicine

Genetics Home Reference related topics: Granulomatosis with polyangiitis

MedlinePlus related topics: Granulomatosis with Polyangiitis

Genetic and Rare Diseases Information Center resources: Granulomatosis With Polyangiitis Eosinophilic Granulomatosis With Polyangiitis ANCA-associated Vasculitis

U.S. FDA Resources

Groups and Cohorts

Outcome Measures

Primary Outcome Measures :

Discover biomarkers in EGPA capable of measuring disease activity and response to treatment [ Time Frame: Study completion ]

Secondary Outcome Measures :

Measure the predictive value of biomarkers for clinical outcome in EGPA [ Time Frame: Study completion. ]

Biospecimen Retention: Samples With DNA

Blood (serum and plasma), urine, and DNA

Eligibility Criteria

Information from the National Library of Medicine

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Ages Eligible for Study:	Child, Adult, Older Adult
Sexes Eligible for Study:	All
Accepts Healthy Volunteers:	No
Sampling Method:	Non-Probability Sample

Study Population

Individuals with eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Enrollment will be sequential and participants will have disease in various stages and of different duration.

Criteria

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

Contacts and Locations

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00315380

Locations


United States, Massachusetts
Boston University School of Medicine	Recruiting
Boston, Massachusetts, United States, 02118
Contact: Naomi Amudala, NP 617-414-2512 namudala@bu.edu
Principal Investigator: Paul A. Monach, MD, PhD
United States, Minnesota
Mayo Clinic	Recruiting
Rochester, Minnesota, United States, 55905
Contact: Sam Hughes Hughes.Samantha@mayo.edu
Principal Investigator: Ulrich Specks, MD
Principal Investigator: Steven R. Ytterberg, MD
United States, Ohio
Cleveland Clinic Foundation	Recruiting
Cleveland, Ohio, United States, 44195
Contact: Elizabeth Kisela kiselae@ccf.org
Principal Investigator: Carol A. Langford, MD, MHS
United States, Pennsylvania
University of Pennsylvania	Recruiting
Philadelphia, Pennsylvania, United States, 19104
Contact: B.Minal Hatwar Bhagyavati.Hatwar@uphs.upenn.edu
Principal Investigator: Peter Merkel, MD, MPH
University of Pittsburgh	Recruiting
Pittsburgh, Pennsylvania, United States, 15260
Contact: Laurie Hope hopelk@upmc.edu
Principal Investigator: Larry Moreland, MD
United States, Utah
University of Utah	Recruiting
Salt Lake City, Utah, United States, 84112
Contact: Jessica gonzalez jessica.gonzalez@hsc.utah.edu
Principal Investigator: Curry Koening, MD, MHS
Canada, Ontario
St. Joseph's Healthcare	Recruiting
Hamilton, Ontario, Canada
Contact: Sandra Messier 905-522-1155 ext 35873 smessier@stjoes.ca
Principal Investigator: Nader A. Khalidi, MD
Mount Sinai Hospital	Recruiting
Toronto, Ontario, Canada
Contact: Judy Vendramini judy.vendramini@sinaihealthsystem.ca
Principal Investigator: Simon Carette, MD

Sponsors and Collaborators

University of Pennsylvania

Office of Rare Diseases (ORD)

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)

Rare Diseases Clinical Research Network

Investigators


Study Chair:	Peter A. Merkel, MD, MPH	University of Pennsylvania

More Information

Additional Information:

Vasculitis Clinical Research Consortium This link exits the ClinicalTrials.gov site

Rare Diseases Clinical Research Consortium This link exits the ClinicalTrials.gov site

Publications:

Heeringa P, Schreiber A, Falk RJ, Jennette JC. Pathogenesis of pulmonary vasculitis. Semin Respir Crit Care Med. 2004 Oct;25(5):465-74.

Radice A, Sinico RA. Antineutrophil cytoplasmic antibodies (ANCA). Autoimmunity. 2005 Feb;38(1):93-103. Review.

Said G, Lacroix C. Primary and secondary vasculitic neuropathy. J Neurol. 2005 Jun;252(6):633-41. Epub 2005 Apr 5. Review.


Responsible Party:	Peter Merkel, Professor, University of Pennsylvania
ClinicalTrials.gov Identifier:	NCT00315380 History of Changes
Other Study ID Numbers:	VCRC5506 U54AR057319 ( U.S. NIH Grant/Contract )
First Posted:	April 18, 2006 Key Record Dates
Last Update Posted:	April 25, 2018
Last Verified:	April 2018
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD:	No

Keywords provided by Peter Merkel, University of Pennsylvania:


EGPA CSS

Additional relevant MeSH terms:


Churg-Strauss Syndrome Systemic Vasculitis Granulomatosis with Polyangiitis Vasculitis Vascular Diseases Cardiovascular Diseases Lung Diseases, Interstitial Lung Diseases	Respiratory Tract Diseases Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Autoimmune Diseases Immune System Diseases Granuloma Lymphoproliferative Disorders Lymphatic Diseases

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