Stay vigilant and think of Wegener

Introduction:

The diagnosis of Wegener's disease can be very difficult. Chronic sinusitis and co-reaction of the lungs often occur to the ENT doctor.

Material and patient:

Our 45-year old patient has been diagnosed with Samter Triad years ago and had undergone seven functional endoscopic sinus surgeries (FESS) because of recurrent polyposis. Since early 2017, a therapy for progressive neuropathic musculoskeletal pain and trigeminal neuralgia has been started. In the same time she developed exacerbations of a chronic sinusitis and was treated with long-term antibiotic therapy. An increase in the inflammatory values as well as thrombocytosis from 716 10^9/l was noticed. The preoperative computer tomography of the nasal sinuses showed the typical picture of osteitis.

Results:

The last surgery was performed in September 2017. In the course of the operation the endoscopic view of the sinuseis showed a necrotic mucosa with additional purulent secretion. The histopathological assessment revealed the diagnosis of ANCA-associated, necrotizing granulomatosis in the sense of Wegener's disease (granulomatosis with polyangitis, GPA). We initiated a high dose cortisone therapy. With regards to the the infiltration of the pulmonary tract, an immunotherapy based on Rituximab was indicated and closely monitored. This resulted over weeks in a clear improvement of the ENT findings and a regression of neuropathic pain.

Conclusion:

We concluded that the diagnosis of polyposis nasi or Samter trias does not exclude a granulomatosis. Even after years the Wegener's disease can unmask itself and should be thought of as a differential diagnosis.

Publication History

Publication Date:
18 April 2018 (online)