Difficulty of diagnosing Wegener's granulomatosis in the head and neck region.
OBJECTIVE
The objective of this study was to review the various clinical features associated with Wegener's granulomatosis (WG) in the head and neck region and to discuss the difficulty of diagnosing patients with early stage WG.


METHODS
Between January 1998 and August 2007, WG was diagnosed and treated in 16 patients at the Department of Otolaryngology, Hyogo College of Medicine. Clinical and operating records of these patients were analyzed retrospectively. Diagnosis was based on the Japanese criteria proposed by the Japanese Ministry of Health and Welfare in 1998.


RESULTS
Ten patients (62.5%) had a definite diagnosis of WG, and the other six patients (37.5%) had a probable diagnosis of WG. The period from the onset to diagnosis was between 1 month and 30 years. The generalized form of WG was observed in three patients (18.8%), and the limited form of WG was observed in the other 13 patients (81.2%). Nasal, aural, and ophthalmic symptoms were initially presented in 10, 3, and 3 patients, respectively. Cytoplasmic pattern antineutrophil cytoplasmic antibodies (cANCAs) and perinuclear pattern ANCA (pANCA) were positively detected in 68.8% (11/16) and 27.2% (3/11) of the patients, respectively. Five of 14 patients (35.7%) had pathologic features of WG in biopsy samples from the head and neck region. Three patients in whom a diagnosis of WG was difficult are presented, and immediate lessons of our experience were discussed.


CONCLUSIONS
This study emphasized the difficulty of diagnosing WG, particularly at an early stage and when limited to the head and neck region. The biggest challenge faced in diagnosing WG is that it requires a high index of suspicion. When WG was suspected, we should obtain an accurate medical history from patients and repeat serologic and histopathologic examinations.
Tsuzuki
Kenzo
K
Department of Otolaryngology, Hyogo College of Medicine, 1-1 Mukogawa, Nishinomiya, Hyogo 663-8501, Japan. kenzo@hyo-med.ac.jp
Fukazawa
Keijiro
K
Takebayashi
Hironori
H
Hashimoto
Kengo
K
Sakagami
Masafumi
M
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D001799:Blood Sedimentation; D002097:C-Reactive Protein / Q000032:analysis; D002558:Cerebrospinal Fluid Otorrhea / Q000209:etiology; D003389:Cranial Nerve Diseases / Q000209:etiology; D003607:Dacryocystitis / Q000209:etiology; D004172:Diplopia / Q000209:etiology; D004433:Earache / Q000209:etiology; D004844:Epistaxis / Q000209:etiology; D005094:Exophthalmos / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D034381:Hearing Loss / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D015508:Nasal Obstruction / Q000209:etiology; D009666:Nose / Q000002:abnormalities; D012189:Retrospective Studies; D015423:Scleritis / Q000209:etiology; D014012:Tinnitus / Q000209:etiology; D014792:Visual Acuity
D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D002097:C-Reactive Protein