The systemic vasculitides are a group of diseases characterized by severe inflammation of blood vessels leading to arterial stenosis or occlusion, and organ damage or death if untreated. With aggressive immunosuppressive treatment, most patients survive, but subsequent disease course is variable, relapse is common, and long-term morbidity from vasculitis or its treatment is expected. Long-term longitudinal studies are needed to inform and guide clinicians in this era of adequate but imperfect management. There are also significant unmet needs for i) biomarkers of disease activity, risk of relapse, and long-term damage;ii) genetic epidemiology studies to provide insight into pathogenesis and ideas for therapeutics;iii) early-phase clinical trials of novel therapeutics;iv) novel methods to conduct high-quality clinical trials and clinical research studies in these rare diseases;and v) for improved outcome measures, including patient-reported outcomes. The VCRC Longitudinal Studies address all these unmet needs through the multiple research studies associated with these protocols. The Vasculitis Clinical Research Consortium, initiated in 2004 under the auspices of the NIH Rare Diseases Clinical Research Network (RDCRN), encompasses longitudinal cohort studies of six vasculitides [giant cell arteritis (GCA), Takayasu's arteritis (TAK), polyarteritis nodosa (PAN), granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg- Strauss, EGPA)] at leading referral centers in the US and Canada. More than 5000 patients have been enrolled. The VCRC Longitudinal Studies are central to the goals of this RDCRC and serve as the foundation for the exploration of new potential biomarkers of disease, phase II and 111 clinical trials, development of improved outcome measures, training of fellows in vasculitis research, and collaboration with patient support organizations.
The Specific Aims of this project are to 1) support the continued successful conduct of the VCRC Longitudinal Studies of six vasculitides, 2) expand the Longitudinal Studies to include patients with isolated aortitis, and 3) maintain and expand the VCRC Biospecimen and Clinical Data Repositories for use by a broad range of scientists, both within and beyond the core group of Consortium investigators.

Public Health Relevance

The Vasculitis Clinical Research Consortium (VCRC) is an extensive infrastructure that allows experts in multiple centers to collaborate on research in vasculitis, a group of rare and serious diseases that feature inflammatory destruction of blood vessels. The VCRC Longitudinal Studies, in which clinical information on patients is collected for up to 10 years, serve as the foundation for the Consortium's many clinical research studies and clinical trials.

National Institute of Health (NIH)
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Specialized Center--Cooperative Agreements (U54)
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Special Emphasis Panel (ZTR1-CI-8 (01))
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Wang, Yan Z
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University of Pennsylvania
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Kermani, Tanaz A; Sreih, Antoine G; Cuthbertson, David et al. (2018) Evaluation of damage in giant cell arteritis. Rheumatology (Oxford) 57:322-328
Springer, Jason Michael; Monach, Paul; Cuthbertson, David et al. (2018) Serum S100 Proteins as a Marker of Disease Activity in Large Vessel Vasculitis. J Clin Rheumatol 24:393-395
Robson, Joanna C; Dawson, Jill; Cronholm, Peter F et al. (2018) Health-related quality of life in ANCA-associated vasculitis and item generation for a disease-specific patient-reported outcome measure. Patient Relat Outcome Meas 9:17-34
Grayson, Peter C; Eddy, Sean; Taroni, Jaclyn N et al. (2018) Metabolic pathways and immunometabolism in rare kidney diseases. Ann Rheum Dis 77:1226-1233
Barra, Lillian; Borchin, Renée L; Burroughs, Cristina et al. (2018) Impact of vasculitis on employment and income. Clin Exp Rheumatol 36 Suppl 111:58-64
Robson, Joanna C; Dawson, Jill; Doll, Helen et al. (2018) Validation of the ANCA-associated vasculitis patient-reported outcomes (AAV-PRO) questionnaire. Ann Rheum Dis 77:1157-1164
Sreih, Antoine G; Alibaz-Oner, Fatma; Easley, Ebony et al. (2018) Health-related outcomes of importance to patients with Takayasu's arteritis. Clin Exp Rheumatol 36 Suppl 111:51-57
Sreih, Antoine G; Ezzedine, Rana; Leng, Lin et al. (2018) Role of Macrophage Migration Inhibitory Factor in Granulomatosis With Polyangiitis. Arthritis Rheumatol 70:2077-2086
Kermani, Tanaz A; Diab, Sehriban; Sreih, Antoine G et al. (2018) Arterial lesions in giant cell arteritis: A longitudinal study. Semin Arthritis Rheum :
Quinn, Kaitlin A; Ahlman, Mark A; Malayeri, Ashkan A et al. (2018) Comparison of magnetic resonance angiography and 18F-fluorodeoxyglucose positron emission tomography in large-vessel vasculitis. Ann Rheum Dis 77:1165-1171

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