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Case Reports
. 2011:2011:608942.
doi: 10.5402/2011/608942. Epub 2011 Jun 8.

Wegener's granulomatosis presenting with pachymeningitis: clinical and imaging remission by rituximab

Søren Andreas Just  1 , John Bonde Knudsen, Mie Kiszka Nielsen, Peter Junker
Affiliations
Case Reports

Wegener's granulomatosis presenting with pachymeningitis: clinical and imaging remission by rituximab

Søren Andreas Just et al. ISRN Rheumatol. 2011.

Abstract

A 27-year-old woman was admitted for intractable right-sided neck, ear, and jaw pain with gradual development of tinnitus and hearing loss. A cerebral MRI showed meningo-dural enhancement, and additional diagnostic workup revealed a right pulmonary infiltrate and positive PR-3 ANCA. Biopsies from nasal mucosa and lung showed chronic inflammation with granuloma formation. Based on these findings the patient was diagnosed with Wegener's granulomatosis with pachymeningitis. There was no clinical response to oral Prednisolone and Cyclophosphamide, but complete clinical and imaging remission was achieved by adding Rituximab.

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Figures

Figure 1
Figure 1
(a) T1W gadolinium-enhanced MRI 12.20.2006 showing meningeal thickening with enhancement on the dorsal and lateral aspects of the clivus close to the transverse sinus (arrows). (b) T1W gadolinium-enhanced MRI 06.08.2007. The meningeal thickening on the back of clivus has completely regressed, but the thickening on the right side near the transverse sinus remains visible. (c) T1W gadolinium-enhanced MRI 02.12.2009. Meningeal thickening and enhancement have resolved completely.
Figure 2
Figure 2
Cumulated annual Prednisolone and Cyclophosphamide (CYC) dose (gram). Rituximab (RTX) treatment cycles are indicated by arrows.
See this image and copyright information in PMC

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