gms | German Medical Science

The aim of our poster is to present several cases of Wegener’s granulomatosis (WG) patients with head and neck manifestations including epistaxis, nasal crusting, prolonged sinus infections with atypical microbiological causes and nasal polyposis.

Materials and methods: These WG patients were referred to our clinic in 2016 for evaluation on the account of their complaints. Among the used methods were full ENT examinations, CT scan of neck and mediastinum, fibrolaryngoscopy, serological testing for ANCA antibodies.

Discussion/conclusion: WG is an idiopathic systemic inflammatory disease typically presenting with vasculitis affecting the upper and lower respiratory tracts and the kidneys. The classical histopathologic features include granulomatous inflammation, necrosis, and/or vasculitis. The initial symptoms typically involve the ENT region. The nose and sinuses are the most frequently affected sites in the head and neck.

The differential diagnosis of ENT disease includes chronic infections (e.g., tuberculous, fungal, and syphilitic), malignancy (e.g., lymphoma), sarcoidosis, Churg-Strauss syndrome. The differential diagnosis of septal perforations associated with WG includes sarcoidosis, cocaine use, SLE, extranodal nasal lymphoma, lymphomatoid granulomatosis (LYG), and excessive use of intranasal corticosteroids.

WG is commonly associated with PR3-ANCA and is highly responsive to treatment with GC and cytotoxic agents. Local treatment for disease involving the head and neck region is often necessary. Empiric experience has led to recommendations to maximize upper airway hygiene to minimize obstruction from crusts and other debris, and diminish colonization with infectious agents.

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