VCRC Genetic Repository One-Time DNA Protocol

Status: Recruiting
Location: See all (14) locations...
Study Type: Observational
SUMMARY

The purpose of this study is to identify genes that increase the risk of developing vasculitis, a group of severe diseases that feature inflammation of blood vessels. Results of these studies will provide vasculitis researchers with insight into the causes of these diseases and generate new ideas for diagnostic tests and therapies, and will be of great interest to the larger communities of researchers investigating vasculitis and other autoimmune, inflammatory, and vascular diseases.

Eligibility
Participation Requirements
Sex: All
Minimum Age: 7
Healthy Volunteers: No
View:

• Diagnostic criteria for Giant Cell Arteritis Age at disease onset >50 years (required)

⁃ New onset or new type of localized pain in the head

⁃ Temporal artery abnormality (i.e. temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries)

⁃ ESR of >40mm in the first hour by the Westergren method

⁃ Abnormal artery biopsy (i.e. temporal artery biopsy showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells)

⁃ Large Vessel Vasculitis (LVV) by angiogram or biopsy not explained by something else

• Diagnostic criteria for Takayasu's Arteritis

⁃ Age at disease onset <50 years

⁃ Claudication of extremities

⁃ Decreased brachial artery pulse (one or both arteries)

⁃ Blood pressure difference of >10mm Hg between the arms

⁃ Bruit over subclavian arteries or aorta

⁃ Arteriogram abnormalities compatible with TAK (includes conventional dye angiography or MR angiography or CT angiography)

• Diagnostic criteria for Polyarteritis Nodosa Major criteria (not explained by other causes) felt by investigator to be due to vasculitis

⁃ Arteriographic abnormality

⁃ Presence of granulocyte or mixed leukocyte infiltrate in an arterial wall on biopsy

⁃ Mononeuropathy or polyneuropathy

⁃ Minor criteria (not explained by other causes) felt by investigator to be due to vasculitis

⁃ Weight loss > 4 kg

⁃ Livedo reticularis, cutaneous ulcerations, or skin nodules

⁃ Testicular pain or tenderness

⁃ Myalgias

⁃ Diastolic blood pressure > 90 mm Hg

⁃ Elevated BUN or serum creatinine levels

⁃ Ischemic abdominal pain

⁃ Isolated cutaneous Polyarteritis Nodosa 1. Biopsy-proven cutaneous PAN

• Diagnostic criteria for Granulomatosis with Polyangiitis (Wegener's) (GPA) and Microscopic Polyangitis (MPA)

⁃ Diagnosis of GPA or MPA. Widely accepted diagnostic criteria, as opposed to classification criteria or definitions, have not been developed for GPA & MPA.

⁃ For diagnosis of GPA meets at least 2 of the following 5 modified ACR criteria:

⁃ Nasal or oral inflammation with oral ulcers or nasal discharge with pus or blood

⁃ Abnormal chest radiograph with nodules, fixed infiltrates, or cavities

⁃ Urinary sediment with microhematuria or red cell casts

⁃ Granulomatous inflammation within the wall of an artery or in the perivascular area on biopsy

⁃ Antineutrophil cytoplasmic antibody (ANCA) positive by enzyme immunoassay for either PR3- or MPO-ANCA

⁃ For diagnosis of MPA, meets the Chapel Hill Consensus Conference Definition for MPA:

⁃ Necrotizing vasculitis, with few or no immune deposits, that affects small vessels (i.e., capillaries, venules, arterioles)

⁃ Necrotizing arteritis involving small- and medium-sized arteries may be present

⁃ Necrotizing glomerulonephritis is very common

⁃ Pulmonary capillaritis often occurs

• Diagnostic criteria for Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

⁃ Asthma

⁃ Peak peripheral blood eosinophilia of >10% of total WBC

⁃ Peripheral neuropathy attributable to vasculitis

⁃ Transient pulmonary infiltrates on chest imaging studies

⁃ Paranasal sinus abnormalities or nasal polyposis

⁃ Eosinophilic inflammation on tissue biopsy

⁃ If patients have 4 of the above 6 criteria but lack clearcut documentation of small vessel vasculitis, they are also eligible for enrollment.

Locations
United States
California
Cedars-Sinai Medical Center
Completed
Los Angeles
University of California, San Francisco
Completed
San Francisco
Illinois
Northwestern University
Recruiting
Chicago
Kansas
University of Kansas Medical Center
Completed
Kansas City
Michigan
University of Michigan
Completed
Ann Arbor
Minnesota
Mayo Clinic
Completed
Rochester
New York
Hospital for Special Surgery
Completed
New York
Ohio
Cleveland Clinic
Recruiting
Cleveland
Pennsylvania
University of Pennsylvania
Recruiting
Philadelphia
University of Pittsburgh
Completed
Pittsburgh
Utah
University of Utah
Completed
Salt Lake City
Other Locations
Canada
St. Joseph's Healthcare
Recruiting
Hamilton
Mount Sinai Hospital
Recruiting
Toronto
Turkey
Istanbul University
Completed
Istanbul
Contact Information
Primary
Carol McAlear, MA
cmcalear@upenn.edu
Time Frame
Start Date: October 2010
Estimated Completion Date: August 2027
Participants
Target number of participants: 1000
Sponsors
Leads: University of Pennsylvania
Collaborators: Office of Rare Diseases (ORD), Rare Diseases Clinical Research Network, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)

This content was sourced from clinicaltrials.gov