Difficulty of diagnosing Wegener's granulomatosis in the head and neck region
Introduction
Wegener's granulomatosis (WG) is an idiopathic systemic disease [1] primarily characterized by vasculitis of small vessels that produces necrotizing granulomas. There are three classic pathologic features of WG—vasculitis of small arteries and veins, infiltrations of giant cells, and epithelioid cell granulomas. Of these vasculitis is thought to be strongly associated with antineutrophil cytoplasmic antibodies (ANCAs). The association with ANCA suggests that the vasculitis of WG constitutes an autoimmune disease [2]. Cytoplasmic pattern ANCA (cANCA) with antigen specificity for proteinase 3 (PR3) is a very sensitive serologic marker for WG [3], [4]. Perinuclear pattern ANCA (pANCA) with antigen specificity for myleoperoxidase (MPO) is also a marker of vasculitis [5].
Virtually any organ system can be affected by WG. The clinical onset is various and non-specific. To clarify the complicated features associated with WG, a classification system based on E (ear, nose, throat, and eye), L (lung), and K (kidney) was proposed [6]. WG was expediently divided into a limited form (e.g., E alone, L alone, and EL) and a generalized form (ELK). Many patients with WG predominantly develop the disease via lesions of the upper airway tract. In particular, the nasal mucosa may be the initial region of development [7], and patients with WG often consult otolaryngologists. Therefore, otolaryngologists play an essential role in the treatment of WG.
This study analyzed the clinical profile of WG in the head and neck region. We present patients in whom WG was clinically difficult to be diagnosed in the early stage and discuss the important lessons, which should not be overlooked.
Section snippets
Patients
Between January 1998 and August 2007, WG was primarily diagnosed and treated in 16 patients (4 males and 12 females) aged 15–76 years (mean age: 57.7 ± 13.6 years) at the Department of Otolaryngology, Hyogo College of Medicine. This study involved a retrospective analysis of clinical and operating records. The patients were followed up for between 9 months and 32 years (mean period: 73.8 ± 23.2 months) after diagnosis.
Diagnosis
Clinical features were carefully documented in each case, and hematologic
Patients with WG
Based on the Japanese criteria, 10 patients (62.5%) had a definite diagnosis of WG, and the other 6 patients (37.5%) had a probable diagnosis of WG (Table 1). The period of time from the onset to diagnosis was between 1 month and 30 years. The generalized form of WG was observed in three patients (18.8%). All three patients with the generalized form had a diagnosis of definite WG. The other 13 patients (81.2%) had the limited form of WG. In the limited form, 7 patients had a definite diagnosis
Discussion
We presented the clinical profiles of patients in whom it was difficult to diagnose WG. We based our diagnoses on the criteria of Ministry of Health, Labor and Welfare of Japan (1998). The criteria substantially consider systemic clinical features, typical pathologic features, and cANCA. It is unusual for all patients to meet all criteria, even if WG present. Very few cases exhibited all three classical pathologic features. The low diagnostic ratio on biopsy also makes the WG diagnosis
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