J Rheum Dis.  2013 Jun;20(3):181-185. 10.4078/jrd.2013.20.3.181.

A Case of Wegener's Granulomatosis with Central Nervous System Involvement Mimicking Lung Cancer with Brain Metastasis

• Park JH
• Lee YH
• Ji JD
• Song GG
• Lee SW
• Yoo SH
• Ryu JY
• Kim HR
• Kang KH
• Kang SH
• Kim SW
• Choi SJ

• KMID: 2222758
• DOI: http://doi.org/10.4078/jrd.2013.20.3.181

Abstract

Wegener's granulomatosis (WG) classically consists of necrotizing granulomatous inflammation of the upper and/or lower respiratory tract, necrotizing glomerulonephritis, and an autoimmune necrotizing systemic vasculitis affecting predominantly small vessels. We report a case of WG with central nervous system (CNS) involvement. WG is being diagnosed through pulmonary nodule biopsy. A small nodular lesion in the left posterior basal ganglia of brain being highly suspicious for granulomatosis was detected by MRI. After IV pulse cyclophosphamide and oral corticosteroid treatment for over 4 months, clinical manifestations and CNS lesions in brain MRI is improved. WG might have multiple granulomatous lesions which could be misdiagnosed due to malignancy. CNS involvement in WG is rare but careful evaluation is necessary when there are suspicious symptoms or lesions in CNS.

Keyword

Wegener's granulomatosis; Central nervous system involvement; Antineutrophil cytoplasmic antibody-associated vasculitis

MeSH Terms

Basal Ganglia
Biopsy
Brain
Central Nervous System
Cyclophosphamide
Glomerulonephritis
Inflammation
Lung
Lung Neoplasms
Neoplasm Metastasis
Respiratory System
Systemic Vasculitis
Wegener Granulomatosis
Cyclophosphamide

Figure

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  Figure 1. Chest X ray (PA and lateral view) shows nodular opa-cities and cavitary lesions in both upper lung fields and patchy opa-cities in both lower lung fields.

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  Figure 2. Chest CT shows multiple cavities and nodules in both lung fields and focal consolidation is also noted in upper right lobe.

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  Figure 3. Hematoxylin and eosin stain of a lung nodule. Tissue obtained from VATS biopsy showed giant cells (black arrow) and necrotizing granulomatous vasculitis (white arrow).

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  Figure 4. 18FDG PET-CT (A) Multiple hyper-metabolic lesions in the nasal septum and in both lungs with similar metabolisms (B) Multiple hyper-metabolic lesions in the nasal septum with extension into the adjacent nasal mucosa with similar metabolisms to those of the lungs.18 FDG PET-CT (A)

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  Figure 5. T1-enhanced MR images before (A, C) and after (B, D) four times cyclophosphamide pulse therapy. Arrow indicates an 8.2 mm- sized rim-enhancing small nodule in the left posterior basal ganglia mimicking metastatic cancer.

Reference

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