Otolaryngologic manifestations of small vessel vasculitis George Metaxaris a , Emmanuel P. Prokopakis b, *, Alexander D. Karatzanis b , George Sakelaris a , Panagiotis Heras a , George A. Velegrakis b , Emmanuel S. Helidonis b a Department of Pathology, Kos General Hospital, Kos, Greece b Department of Otolaryngology, University of Crete School of Medicine, Panepistimiou Avenue, 71500 Heraklion, Crete, Greece Received 18 February 2002; received in revised form 1 July 2002; accepted 19 July 2002 Abstract Objective: The aim of the present study is to review the clinical manifestations associated with small vessel vasculitis (SVV) as they pertain to the head and neck region. Methods: A retrospective analysis was performed, including 34 individuals that filled the American College of Rheumatology criteria for the diagnosis of necrotizing vasculitis. Seven patients were classified as suffering from Wegener’s granulomatosis (WG), 18 from microscopic polyangitis (MPA), and the remaining 19 were unclassified (unclassified small vessel vasculitis, USVV). Results: The percentage of ENT manifestations in the early clinical picture of WG patients was 86%, dropping to 44.5 and 22% for MPA and USVV patients, respectively. The overall percentage of ENT manifestations for SVV patients in their initial clinical profile was 47%. Conclusion: The results of our investigation highlight the importance of an ENT clinical examination as a guide for diagnosis of an important percentage of SVV patients. # 2002 Published by Elsevier Science Ireland Ltd. Keywords: Small vessel vasculitis; Wegener’s granulomatosis; ENT manifestations 1. Introduction Vasculitis is a clinicopathological entity characterized by inflammation and necrosis of blood vessels. This heterogeneous vasculitis group commonly referred to as small vessel vasculitis (SVV) includes Wegener’s granu- lomatosis (WG), as well as Polyarteritis Nodosa, and its subgroups: microscopic polyangitis (MPA) or polyangi- tis and Churg  /Strauss syndrome. SVV mainly affects small sized blood vessels [1  /5]. antineutrophil cytoplas- mic antibodies (ANCA) composes a serologic marker common to all SVV entities, which share the same hypothesized autoimmune etiology. In accordance to ANCA, these vasculitis syndromes are distinguished in P-ANCA and C-ANCA positive, while a percentage remains unclassified (ANCA negative) [6  /10]. Patients’ response to treatment and prognosis is related to the timing of diagnosis [11 /14]. Although the final diag- nosis of necrotizing vasculitis relies on laboratory and pathological findings, clinical findings are of equal importance. Therefore, request for the earliest and commonest clinical manifestations of necrotizing vascu- litis is a key issue to early diagnosis. It is also well known that upper airway manifestations are common features in SVV and particularly in WG, where they occur in greater than 70% of patients at onset. The aim of the present study is to adress the otolaryngologic manifestations associated with SVV. This will allow for an Otorhinolaryngologist to ensure more accurate diagnosis and provide better treatment [15  /17]. 2. Patients and methods This is a retrospective analysis of 34 consecutive individuals filling the American College of Rheumatol- ogy criteria for the diagnosis of necrotizing vasculitis [4,18,19]. According to the Chapel Hill Consensus Conference criteria, seven patients were classified as suffering from WG, 18 from MPA, and the remaining nine were unclassified (unclassified small vessel vasculi- tis, USVV) [1]. * Corresponding author. Tel.:  /30-8139-2348; fax:  /30-8154-2106 E-mail address: epro@med.uoc.gr (E.P. Prokopakis). Auris, Nasus, Larynx 29 (2002) 353 /356 www.elsevier.com/locate/anl 0385-8146/02/$ - see front matter # 2002 Published by Elsevier Science Ireland Ltd. PII:S0385-8146(02)00062-7