The Future of Damage Assessment in Vasculitis

PHILIP SEO, RAASHID A. LUQMANI, OLIVER FLOSSMANN, BERNHARD HELLMICH, KAREN HERLYN, GARY S. HOFFMAN, DAVID JAYNE, CEES G.M. KALLENBERG, CAROL A. LANGFORD, ALFRED MAHR, ERIC L. MATTESON, CHETAN B. MUKHTYAR, TUHINA NEOGI, ABRAHAM RUTGERS, ULRICH SPECKS, JOHN H. STONE, STEVEN R. YTTERBERG, and PETER A. MERKEL

ABSTRACT.

Damage denotes the aspects of chronic disease that do not reverse with therapy. This concept is particularly important for the primary systemic vasculitides, since the careful differentiation between activity and damage may help avoid unnecessary exposure to cytotoxic medications. Damage significantly influences both longterm prognosis and quality of life. Because the primary systemic vasculitides have diverse manifestations, the use of a damage assessment instrument is crucial to ensure reproducibility. The Vasculitis Damage Index (VDI) is the only validated measure for damage assessment in vasculitis. Use of the VDI in recent clinical trials has shown that it may not adequately determine the full spectrum of damage experienced by patients with vasculitis of small- and medium-size vessels. We propose reexamining the way in which damage is assessed, focusing on vasculitides of small- and medium-size vessels, and outline an initiative to create a substantially revised and improved damage assessment instrument using data-driven approaches. This initiative is part of a larger international effort to create a unified approach to disease assessment for the primary systemic vasculitides. (J Rheumatol 2007;34:1357-71)

Key Indexing Terms:

VASCULITIS
OUTCOMES
DAMAGE

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From the Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA; Department of Rheumatology, Botnar Research Centre, University of Oxford, Oxford, England; Vasculitis Unit, Addenbrooke's Hospital, Cambridge, England; Department of Rheumatology, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Germany; Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Lerner College of Medicine, Cleveland, Ohio, USA; Renal Unit, Department of Medicine, School of Clinical Medicine, University of Cambridge, Cambridge, England; Department of Clinical Immunology, University Medical Center Groningen and University of Groningen, The Netherlands; Vasculitis Center, Section of Rheumatology, Clinical Epidemiology Unit, Boston University School of Medicine, Boston, Massachusetts, USA; and Division of Rheumatology and Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.

The Vasculitis Clinical Research Consortium (VCRC) is part of the National Institutes of Health Rare Diseases Clinical Research Network (http://rarediseasesnetwork.org/vcrc). Supported by grant U54 RR019497 from the NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) and the National Center for Research Resources (NCRR). Dr. Seo is also supported by NIH/NIAMS grant K23 AR052820; Dr. Merkel is also supported by NIH/NIAMS grant K24 AR2224.

P. Seo, MD, MHS, Assistant Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine; R.A. Luqmani, DM, FRCP, Consultant Rheumatologist, Senior Lecturer in Rheumatology, Department of Rheumatology, Botnar Research Centre, University of Oxford; O. Flossmann, MRCP, Specialist Registrar in Nephrology, Vasculitis Unit, Addenbrooke's Hospital, Cambridge, UK; B. Hellmich, MD, PhD, Vice Chairman, Department of Rheumatology, Universitätsklinikum Schleswig-Holstein; K. Herlyn, MD, MPH, Department of Rheumatology, Universitaetsklinikum Schleswig-Holstein; G.S. Hoffman, MD, MS, Professor, Chairman, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Lerner College of Medicine; D. Jayne, MD, FRCP, Renal Unit, Department of Medicine, School of Clinical Medicine, University of Cambridge;
C.G.M. Kallenberg, MD, PhD, Professor in Medicine, Head, Department of Clinical Immunology, University Medical Center Groningen and University of Groningen; C.A. Langford, MD, MHS, Associate Professor, Director, Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Lerner College of Medicine; A. Mahr, MD, MPH, Research Associate, Vasculitis Center, Section of Rheumatology, Clinical Epidemiology Unit, Boston University School of Medicine; E.L. Matteson, MD, MPH, Professor of Medicine, Division of Rheumatology, Mayo Clinic College of Medicine; C.B. Mukhtyar, DM, MRCP, Research Fellow, Botnar Research Centre, Institute of Musculoskeletal Sciences, University of Oxford; T. Neogi, MD, FRCPC, Assistant Professor of Medicine, Division of Rheumatology, Clinical Epidemiology Unit, Boston University School of Medicine; A. Rutgers, MD, PhD, Fellow, Department of Clinical Immunology, University Medical Center Groningen and University of Groningen; U. Specks, MD, Professor of Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine; J.H. Stone, MD, MPH, Associate Professor of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine; S.R. Ytterberg, MD, Associate Professor of Medicine, Division of Rheumatology, Mayo Clinic College of Medicine; P.A. Merkel, MD, MPH, Associate Professor of Medicine, Director, Vasculitis Center, Section of Rheumatology and Clinical Epidemiology Unit, Boston University School of Medicine.

Address reprints requests to Dr. P.A. Merkel, Vasculitis Center, E5, Boston University School of Medicine, 715 Albany Street, Boston, MA 02118. E-mail: pmerkel@bu.edu

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