Localised Wegener’s Granulomatosis in Otolaryngology: A Review of Six Cases

Abstract

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.

Manifestações otorrinolaringológicas nas doenças reumáticas auto-imunes

“…Quando episódios de sinusite persistentes, recorrentes ou atípicos ocorrem, uma avaliação mais extensa incluindo raio X de tórax, medida da creatinina sérica, ANCA, urinálise e, finalmente, a biópsia do sítio envolvido deve ser considerada (23) . Quadro de GW localizada sem envolvimento pulmonar ou renal, mas ANCA positivo e com histologia compatível tem sido reconhecido como um subtipo distinto da doença (24) . Além disso realça a predileção da GW pela região da cabeça e pescoço, denotando as manifestações freqüentes de cada paciente quanto ao aspecto otorrinolaringológico e o papel crucial da alta suspeita clínica e confirmação sorológica para diagnóstico e tratamento precoces (24) .…”

Management and Follow-up of Localized Wegener's Granulomatosis: A Review of Five Cases

“…WG occurs in two forms: as a disseminated disease mainly involving the respiratory tracts and kidneys and in the localized form in which the kidneys are spared but any other system or site can be affected (6). In both forms, WG may present initially with head and neck signs and symptoms.…”

Rhinochirurgie bei Systemerkrankungen

“…Klassischerweise sind der obere Respirationstrakt, die Lungen und die Nieren betroff en. Bei etwa 10 -15 % der Erkrankten kann sich der M. Wegener jedoch auch nur lokal in der Kopf-Hals-Region manifestieren (lokalisierter M. Wegener) [39] . Die Diagnose kann anhand der Kriterien des American College of Rheumatology gestellt werden [40] .…”