Wegener's granulomatosis triggered by infection?
Wegener's granulomatosis is a systemic disease of unknown origin, although recent studies suggest that auto-immune mechanisms and infection play a role in the pathogenesis of this disease. Wegener is characterized by a necrotizing vasculitis involving the lungs (pulmonary infiltrates), the upper airways and the kidneys (rapidly progressive glomerulonephritis). We present a case of a male patient admitted because of progressive deterioration of the general condition with weight loss, a unilateral neck mass, unilateral purulent rhinorrea and fever. CT-scan evaluation demonstrated a unilateral expanding mass in the sing-nasal cavity, obliterating the ethmoid complex. MRI revealed signs of intracranial inflammatory reaction and onset of absedation. A malignancy was suspected but a diagnosis of Wegener's granulomatosis was established based on histologic criteria (nasal biopsy) and a positive titer for anti-cytoplasmic antibodies (cANCA). During follow-up, nasal carriage of Staphyloccocus Aureus could be documented. An overview of Wegener's granulomatosis will be provided with emphasis on the potential role of acute infections as a trigger for Wegener's granulomatosis and the head and neck manifestations.
Department of Otorhinolaryngology, Head & Neck Surgery, University Hospital Antwerp, Belgium.
A
A
Boudewyns
J
J
Verbelen
E
E
Koekelkoren
J
J
Van Offel
P
P
Van de Heyning
D000208:Acute Disease; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001706:Biopsy; D002353:Carrier State / Q000175:diagnosis; D005221:Fatigue / Q000382:microbiology; D005334:Fever / Q000382:microbiology; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000175:diagnosis* / Q000276:immunology / Q000382:microbiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009297:Nasal Mucosa / Q000382:microbiology*; D012220:Rhinitis / Q000382:microbiology; D012852:Sinusitis / Q000382:microbiology; D013203:Staphylococcal Infections / Q000150:complications* / Q000175:diagnosis; D013492:Suppuration; D014057:Tomography, X-Ray Computed; D015431:Weight Loss
D019268:Antibodies, Antineutrophil Cytoplasmic