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

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.

Uncommon Presentation of Systemic Lupus Erythematosus: Intracranial Mass Lesions as the First Manifestation

“…Although new classification criteria including 2012 SLICC and EULAR/ ACR-2019 criteria are more sensitive, patients still experience diagnostic delays [ 5 , 9 ]. In 2023, a rare case of cerebral vasculitis mimicking a brain tumor in a woman with SLE was reported [ 10 ]. Despite a previous definitive diagnosis of lupus nephritis, this patient underwent a thorough work-up following the development of atypical neurological complications, and intracranial tumor-like manifestations were eventually attributed to SLE [ 10 ].…”

“…In 2023, a rare case of cerebral vasculitis mimicking a brain tumor in a woman with SLE was reported [ 10 ]. Despite a previous definitive diagnosis of lupus nephritis, this patient underwent a thorough work-up following the development of atypical neurological complications, and intracranial tumor-like manifestations were eventually attributed to SLE [ 10 ]. Our patient was different in that there was no previous history of lupus, which led to delayed screening for SLE and the initiation of immunotherapy without a clear basis for diagnosing tumor or infection.…”