CASE REPORT Julio A Chirinos Æ Leonardo J. Tamariz Æ Gilberto Lopes Freddy Del Carpio Æ Xiaohong Zhang Æ Clara Milikowski Daniel M. Lichtstein Large vessel involvement in ANCA-associated vasculitides: report of a case and review of the literature Received: 9 May 2003 / Accepted: 12 September 2003 / Published online: 13 January 2004 Ó Clinical Rheumatology 2004 Abstract Vasculitides are currently classified according to the size of the vessels involved and characteristic clinical and histopathologic findings. Antineutrophil cytoplas- mic antibodies (ANCA) and other serologic tests have been used to further characterize small vessel vasculiti- des. Large vessel involvement in ANCA-associated small vessel vasculitides has been overlooked in the medical literature. Here, we report a case of fatal aortitis and aortic dissection in a patient with microscopic polyan- giitis and review reported cases of large vessel involve- ment in ANCA-associated vasculitides since 1990. We have attempted to characterize this subgroup of patients. Large vessel disease in ANCA-associated vasculitis may present as stenosing large vessel arteritis, aneurysmal disease, aortic dissection, aortic rupture, aortic regurgi- tation, and death. Prominent perivascular inflammation may present as mediastinal, cervical or abdominal soft tissue masses. ANCA-associated large vessel disease should be considered in the differential diagnosis of these disorders. The epidemiologic, clinical and pathologic characteristics of these patients differ from those of the well-defined large vessel vasculitides such as giant cell (temporal) arteritis or Takayasu’s arteritis. We suggest that large vessel involvement is part of the spectrum of ANCA-associated vasculitis rather than an overlap with other large vessel vasculitides. It occurs in both mye- loperoxidase- and proteinase 3-positive patients with ei- ther Wegener’s granulomatosis or microscopic polyangiitis, but has not been reported in Churg–Strauss syndrome. Large vessel vasculitis can precede small ves- sel vasculitis or occur in the absence of small vessel involvement. We hope this report will contribute to the ongoing development of classification systems for the vasculitic syndromes. Keywords Antineutrophil cytoplasmic antibodies Æ Aortic dissection Æ Aortitis Æ Classification Æ Vasculitis Abbreviations ANCA Antineutrophil cytoplasmic anti- bodies Æ CSS Churg–Strauss syndrome Æ ESR Erythro- cyte sedimentation rate Æ MPA Microscopic polyangiitis Æ MPO Myeloperoxidase Æ PR3 Proteinase 3 Æ SVV Small vessel vasculitis Æ WG Wegener’s granulomatosis Introduction Vasculitis comprises a broad group of syndromes char- acterized by inflammation of the blood vessel walls [1, 2, 3]. The distribution of blood vessel involvement serves, in part, as the basis for the Chapel Hill consensus con- ference classification system for the systemic vasculitides [1, 2, 3]. Antineutrophil cytoplasmic antibodies (ANCA) have been used to further characterize small vessel vas- culitides. Wegener’s granulomatosis (WG), microscopic polyangiitis (MPA), the Churg–Strauss syndrome (CSS) and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are associated with ANCA directed against either proteinase 3 (PR 3) or myelop- eroxidase (MPO), and some authors have grouped these diseases under the common heading of ANCA-associ- ated vasculitides [4, 5, 6]. The first three represent sys- temic vasculitides, whereas the latter is confined to the kidneys [4, 5, 6, 7]. Large vessel vasculitides encompass fewer distinct clinical syndromes and entities than do vasculitides of the medium or small vessels. Only two major primary Clin Rheumatol (2004) 23: 152–159 DOI 10.1007/s10067-003-0816-0 J. A. Chirinos Æ G. Lopes Æ F. Del Carpio Æ D. M. Lichtstein Department of Medicine, School of Medicine, University of Miami, Miami, USA X. Zhang Æ C. Milikowski Department of Pathology, School of Medicine, University of Miami, Miami, USA L. J. Tamariz (&) Department of Medicine, School of Medicine, Johns Hopkins University, Johns Hopkins Medical Institutions, 2024 East Monument Street, Rm 2516, Baltimore, MD 21205, USA E-mail: ltamari1@jhmi.edu Tel.: +1 -410-5028897 Fax: +1-410-6140588