J Korean Rheum Assoc.  2010 Dec;17(4):406-411. 10.4078/jkra.2010.17.4.406.

A Case of Wegener's Granulomatosis with Periaortitis and Pachymeningitis

• Chang SH
• Kim HW
• Yun CY
• Kang EH
• Lee EY
• Lee YJ
• Lee EB
• Song YW

• KMID: 2201899
• DOI: http://doi.org/10.4078/jkra.2010.17.4.406

Abstract

Wegener's grandulomatosis (WG) is a systemic small-vessel vasculitis characterized by necrotizing granulomatous lesions and anti-neutrophil cytoplasmic antibodies (ANCA). WG frequently affects the upper and lower respiratory tracts and kidney but involvement of the pachymeninges or large vessels has rarely been reported. We report the first case of WG with periaortitis and pachymeningitis in Korea. A 57-year-old female complained of a stabbing headache, paresthesia below the thoracic 4th nerve level, and lower extremity weakness. During her course, she developed otitis media with sensorineural hearing loss, nodular scleritis, and microhematuria. A neuroimaging study showed an intradural mass in the area from the cervical 7th spine to the thoracic 5th spine, left cerebral convexity, and the left petrous area. A chest computed tomography scan revealed an enhanced and thickened aortic arch. A dural biopsy showed necrotizing granulomatous inflammation, and anti-MPO ANCA was positive. Under a diagnosis of WG, she received high-dose glucocorticoid and oral cyclophosphamide with improvement.

Keyword

Wegener's granulomatosis; Periaortitis; Pachymeningitis

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Aorta, Thoracic
Biopsy
Cyclophosphamide
Female
Headache Disorders, Primary
Hearing Loss, Sensorineural
Humans
Inflammation
Kidney
Korea
Lower Extremity
Meningitis
Middle Aged
Neuroimaging
Otitis Media
Paresthesia
Respiratory System
Scleritis
Spine
Thorax
Vasculitis
Wegener Granulomatosis
Antibodies, Antineutrophil Cytoplasmic
Cyclophosphamide

Figure

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  Fig. 1. An intradural and extramedullary mass (white arrow) at the lower cervical to upper thoracic spine area was shown in the sagittal T2-weighted spinal magnetic resonance images taken 5 months before the Wegener's granulomatosis diagnosis (A), at the diagnosis (B), and 9 months after immunosuppressive treatment (C). The T2 high signal intensity changes in the spinal cord are suggestive of myelopathy secondary to cord compression.

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  Fig. 2. A chest computed tomography scan showed localized wall enhancement with peripheral onion-shaped density at the aortic arch level (A). This abnormally enhanced lesion was much improved after 9 months of immunosuppressive treatment (B).

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  Fig. 3. Coronal and axial T2-weighted brain magnetic resonance images revealed enhancement and thickening of the meninges (white arrows) at the left frontoparietotemporal convexity and left petrous area at diagnosis (A) and 9 months after treatment (B).

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  Fig. 4. Histopathology of the dural biopsy showed necrotizing granulomatous inflammation and infiltration of plasma cells and lymphocytes (A, hematoxylin and eosin, ×200). Immunohistochemical staining for an endothelial cell marker (CD31) revealed a damaged vessel with an obliterated lumen surrounded by granulomatous inflammation (B, ×400).

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