Localised Wegener's granulomatosis in otolaryngology: a review of six cases.
Wegener's granulomatosis (WG) is a necrotising granulomatous disease affecting the upper and/or lower respiratory tracts and is associated with focal glomerulonephritis. Formerly believed to be a multisystem disease, a localised form (LWG) is now recognised as a distinct subtype. We describe 6 cases of LWG with no renal or pulmonary involvement detected at the presentation or during follow-up. The total follow-up period ranged from 3 to 7 years in 5 cases. The diagnosis was based on clinical features, antineutrophil cytoplasmic antibody test and histological findings (necrotising granulomatous vasculitis, epithelioid granulomas with varying degrees of chronic inflammatory cells). All patients responded to standard immunosuppressive treatment. Our cases highlight the predilection of LWG for the head and neck region and hence these patients frequently present in the ENT departments. LWG has been discussed as a subtype of WG with a better prognosis and the previous literature has been reviewed on this subject. A high index of suspicion helped by serology and histology enables an early diagnosis, and commencement of proper treatment can prevent the irreversible destructive lesions.
Ahmad
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Russells Hall Hospital, Dudley Group of Hospitals, Dudley, UK. Iahmed6188@aol.com
Lee
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WC
Nagendran
V
V
Wilson
F
F
Shortridge
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RT
D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism*; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D001799:Blood Sedimentation; D002097:C-Reactive Protein / Q000378:metabolism; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D010256:Paranasal Sinuses / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D000964:Antimetabolites, Antineoplastic; D018906:Antineoplastic Agents, Alkylating; D003520:Cyclophosphamide; D002097:C-Reactive Protein; D001379:Azathioprine; D008727:Methotrexate