J Rheum Dis.  2023 Jan;30(1):53-57. 10.4078/jrd.22.0022.

Tumor-like Presentation of Cerebral Vasculitis in a Patient With Systemic Lupus Erythematosus: A Biopsy-confirmed Case

• Kim NR
• Kang JW
• Nam EJ

• KMID: 2537495
• DOI: http://doi.org/10.4078/jrd.22.0022

Abstract

Central nervous system (CNS) manifestations of systemic lupus erythematosus (SLE) are diverse and often difficult to distinguish from SLE-unrelated events. CNS vasculitis is a rare manifestation, which is seen in less than 10% of post-mortem studies, and lesions with multifocal cerebral cortical microinfarcts associated with small-vessel vasculitis are the predominant feature. However, CNS vasculitis presenting as a tumor-like mass lesion in SLE has rarely been reported. Herein, we report a case of cerebral vasculitis mimicking a brain tumor in a 39-year-old female with SLE. A biopsy of the brain mass revealed fibrinoid necrosis and leukocytoclastic vasculitis. The neurological deficits and systemic symptoms improved after treatment with corticosteroids and immunosuppressive agents. To the best of our knowledge, there are no reports of biopsy-proven cerebral vasculitis presenting as a brain mass in patients with SLE in Korea.

Keyword

Central nervous system vasculitis; Systemic lupus erythematosus; Tumor; Biopsy

Figure

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  Fig. 1 Light micrograph of renal specimen shows glomeruli with mesangial expansion and segmental endocapillary proliferation (PAS, A: ×100, B: ×200).

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  Fig. 2 (A, B) Initial magnetic resonance imaging (MRI) of the brain. The axial T2-weighted image (T2WI) (A) shows a large intra-axial heterogeneous mass in the left frontal lobe with perilesional edema. Note that the prominent hemorrhagic component in the mass and other remote lesions of both frontoparietal lobes are revealed in the axial susceptibility-weighted image (SWI) (B). No significant enhancement is seen. (C, D) Follow-up MRIs after four months of immunosuppressive treatment. Axial T2WI (C) and SWI (D) demonstrate decrease in mass size, perilesional edema, and peripheral hemosiderin rim formation, even in non-operated remote lesions. No new lesions were observed.

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  Fig. 3 Histopathology from a biopsy of left frontal brain lesion shows (A) a necrotizing vasculitis with fibrinoid necrosis of vessel wall and (B) leukocytoclastic vasculitis with prominent neutrophil infiltration and adjacent nuclear debris in a perivascular area (H&E, A: ×100, B: ×400).

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