Article Text

  1. M. B. Knauf1,
  2. S. G. West1,
  3. B. K. DeMasters1,
  4. M. Thakor1,
  5. K. D. Deane1
  1. 1University of Colorado at Denver and Health Sciences Center, Denver, CO.


Objective Meningeal involvement is a rare complication of Wegener's granulomatosis (WG). Here we present a case of pachymeningitis in WG and perform a literature review to describe the clinical features and strategies for diagnosis of meningeal disease in WG.

Methods Medline search from 1960 to present was performed and articles in English describing meningeal involvement in WG were reviewed. For the case presentation, pertinent photographs of clinical findings and diagnostic studies were obtained with the patient's permission.

Results A 25-year-old male patient with known limited WG was admitted with new-onset right-sided ear pain, hearing loss, facial droop, diplopia, and difficulty swallowing. His WG had manifested in the past with scleritis, arthritis, sinusitis, and lung disease in the setting of high-titer c-ANCA and anti-proteinase-3 (PR3) reactivity. He was on prednisone and low-dose daily oral cyclophosphamide (75 mg daily). Physical examination revealed right eye proptosis, ptosis, and facial droop, lateral gaze palsy, and deviation of the tongue to the right. C-reactive protein was 22.3 mg/dL. Repeat c-ANCA titers were low titer positive. Spinal fluid was negative for inflammatory cells or infection. Magnetic resonance imaging showed opacification of the right middle ear, right mastoiditis, right-sided occipital-temporal meningeal enhancement, and enhancement of the right lateral extraocular muscles but no significant anterior sinus disease. Biopsy of the right dura overlying the temporal lobe revealed multifocal necrotizing granulomas with vessel destruction and no evidence of infection. He was placed on high-dose intravenous corticosteroids and intravenous cyclophosphamide with gradual clinical improvement.

Conclusion Meningeal involvement in WG typically presents in patients with limited disease. Clinical features include headache, hearing loss, and cranial nerve dysfunction. Neuroimaging can reveal diffuse or focal meningeal inflammation. In up to one-third of WG patients with pachymeningitis, ANCA testing may be negative. As such, meningeal biopsy may be necessary to confirm the diagnosis, especially if infection is a consideration.

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