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03 June 2024: Articles  China (mainland)

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

Mistake in diagnosis, Unusual setting of medical care, Patient complains / malpractice, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

Nannan Dong1ABCDE, Mengqi Niu1ABCDE, Xialing Wang1CDE, Zhenzheng Bai1ACE, Si Tian1CDE, Kebin Zeng1DFG*

DOI: 10.12659/AJCR.942877

Am J Case Rep 2024; 25:e942877

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Abstract

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BACKGROUND: Multi-system damage is a hallmark of systemic lupus erythematosus (SLE), a chronic systemic autoimmune disease. The typical initial symptoms of SLE are arthritis and dermatosis, whereas the presence of intracranial mass lesions as the first manifestation of systemic lupus erythematosus is very rare. This report describes an 18-year-old woman with intracranial mass lesions associated with SLE.

CASE REPORT: An 18-year-old woman was initially admitted to the hospital because of headache for 3 days, weakness in left arm, and blurred vision for 1 day. Magnetic resonance imaging (MRI) of her brain showed multiple abnormal occupying lesions in the right frontoparietal lobe. However, no evidence of tumor or infection was found. One month later, she was readmitted with right limb weakness and aphasia for 1 day. Brain MRI showed obvious and new abnormal signal shadows in both the right parietal lobe and the left frontotemporal parieto-occipital lobes compared with the previous MRI. She responded positively to immunotherapy, which, in a woman of child-bearing age, supports the diagnosis of SLE. Ultimately, the presence of focal neurological symptoms, abnormal autoantibodies (such as antinuclear antibodies, anti-dsDNA antibodies, anti-SSA autoantibodies, and anti-ribosomal P protein antibodies), as well as her positive response to immunotherapy, contributed to the diagnosis of SLE with intracranial mass lesions. No recurrence was seen during 1 year of follow-up.

CONCLUSIONS: It is unusual for SLE to present with intracranial mass lesions as the initial symptoms. The pathogenesis of the neurological symptoms of the patient may be small vessel thrombosis or vasculitis leading to cerebral mass-like necrosis.

Keywords: Magnetic Resonance Imaging, Clinical Medicine, Intracranial Thrombosis, Lupus Vasculitis, Central Nervous System

Introduction

Systemic lupus erythematosus (SLE) is characterized by damage to organs and tissues caused by pathogenic autoantibodies and immune complexes. Neurological and psychiatric manifestations of SLE are collectively referred to as neuropsychiatric SLE (NPSLE) [1]. The typical initial symptoms of SLE are arthritis and dermatosis [2]. The reported prevalence of neuropsychiatric syndromes in SLE is approximately 56% [1], of which 6% to 12% occur in the first year of newly diagnosed SLE [3]. Approximately 20% of NPSLE cases present with neuropsychiatric symptoms as the first manifestation [4]. However, most brain magnetic resonance images do not show any abnormalities or only white matter hyperintensity or atrophy [4]. The most common brain magnetic resonance imaging (MRI) abnormality is cerebrovascular disease, accounting for approximately 8% of neuropsychiatric lupus cases [1]. Intracranial mass lesions are uncommon as the initial presentation of SLE, and these lesions can easily be mistaken for tumors or intracranial infections, thus delaying treatment. The diagnosis of SLE can be challenging, and no single clinical feature or lab abnormality can confirm SLE diagnosis [5]. The American College of Rheumatology (ACR) first developed the SLE classification criteria in 1971. These criteria were further revised by the Systemic Lupus International Collaborating Clinics (SLICC) group in 2012 [5]. In September 2019, the European League Against Rheumatism (EULAR) and the ACR published new criteria for the classification of SLE [5]. The EULAR/ACR-2019 criteria have a specificity of 93.4% and sensitivity of 96.1%, as opposed to the 83.7% specificity and 96.7% sensitivity of the 2012 SLICC classification criteria [5,6]. Here we report a case of a patient who presented with intracranial mass lesions as the first manifestation of SLE, and who was hospitalized twice. The final clinical diagnosis of SLE was made after careful exclusion of other diseases and consideration of the whole clinical presentation as well as serological and imaging tests.

Case Report

FIRST HOSPITALIZATION:

In August 2021, an 18-year-old woman was admitted to the hospital because of headache for 3 days, weakness in her left arm and blurred vision for 1 day. Before this sickness, she was in good health and denied any history of smoking or alcohol or substance abuse. There was no fever. Blood pressure was 94/63 mmHg, and left arm muscle strength was grade IV. Her electroencephalogram (EEG) showed slow and sustained activity on the right side, marked by delta waves (Figure 1A). MRI revealed multiple abnormal lesions in the right frontoparietal lobe (Figure 2A–2D). Magnetic resonance spectroscopy (MRS) did not show a tumor spectrum. No abnormalities were detected in the cerebrospinal fluid (CSF) by multiple laboratory tests, including routine, biochemical, culture, cytology, and second-generation sequencing virus tests. White blood cells, neutrophils, C-reactive protein (CRP), the treponema pallidum particle (TPPA), and syphilis antibodies by enzyme-linked immunosorbent assay (ELISA) were all negative. Mannitol and furosemide were administered to reduce intracranial pressure. After 20 days, the patient’s headache and muscle strength completely improved. A follow-up brain MRI revealed partially absorbed lesions with liquefied necrosis (Figure 2E–2H). MRS revealed a reduced N-acetyl-aspartate (NAA) peak and abnormal lactate peak, suggesting neuronal damage. Subsequently, the patient refused treatment and was discharged for follow-up.

SECOND HOSPITALIZATION:

One month after discharge, the patient was readmitted to the hospital due to symptoms including headache, fever (maximum 39°C), weakness of the right limbs, and aphasia for 1 day. Nervous system examination showed consciousness, mixed aphasia, right central facial paralysis, right limb muscle strength grade 0, left limb muscle strength grade III, and negative pathological signs. EEG showed continuous low voltage in the anterior head leads, most obviously on the right side (Figure 1B). No obvious abnormalities were found in computed tomography angiography and computed tomography venography of the head and neck (Figure 2L–2P). Brain MRI showed that, compared with the previous MRI, there were obvious and new abnormal signal shadows in the right parietal lobe and left frontotemporal and parieto-occipital lobes (Figure 2I–2K). These unique signal shadows may indicate inflammation, although antibody-mediated autoimmune encephalitis antibody testing in CSF revealed no significant findings. However, the patient tested positive for serum antinuclear antibodies at a dilution of 1: 1000, as well as anti-dsDNA, anti-SSA, and anti-ribosomal P protein antibodies. According to a repeat blood test, leukocytes (2.73×106 cells/L), hemoglobin (103 g/L), complement C3 (0.67 g/L) and complement C4 (0.12 g/L) were all decreased. The patient finally met guidelines developed by SLICC and EULAR/ACR indicating a diagnosis of NPSLE, following the exclusion of other potential diseases [5]. The SLE disease activity index (SLEDAI) score of 12 indicated active disease [7]. Dexamethasone 10 mg/d was prescribed, plus an appropriate amount of mannitol for intravenous infusion. The patient’s speech and limb muscle strength improved after 15 days. Brain MRI showed the smaller focus with signs of necrolysis and liquefaction (Figure 2M–2O), indicating the possibility of thrombosis or inflammation in small blood vessels, leading to tissue necrosis. After discharge, she started taking oral prednisone tablets (40 mg per day) and the dosage was gradually reduced during outpatient follow-up. No recurrence was found during 1 year of follow-up. She looks after herself and is now able to interact with people fluently.

Discussion

Neuropsychiatric complications can manifest at any point during the course of SLE, and may even be the initial presentation [4]. In cases where women of reproductive age have unexplained neurological complaints and cranial imaging shows mass lesions, a diagnosis should not be based solely on intracranial occupancy. To avoid treatment delays and rule out other possible disorders, it is critical to establish the diagnosis as soon as possible by testing for autoantibodies related to SLE. Our patient was admitted to the hospital due to non-specific manifestations such as recurring headaches and fever, as well as neurological manifestations such as limb weakness. Dynamic follow-up brain MRI showed that multiple space-occupying lesions involving both cerebral hemispheres appeared in a short period of time. After treatment, the space-occupying effect of the lesion gradually weakened. MRS found no tumor spectrum and no heterotypic cells were found in the CSF, thus ruling out the possibility of tumor. There was no significant increase in inflammatory markers in the blood, and no abnormalities were found in routine, biochemical, culture, and second-generation sequencing virus tests in the CSF. In addition, without anti-infective treatment, the patient’s symptoms improved, so intracranial infectious disease and infection-induced intracranial vasculitis were eliminated. For this patient, despite the lack of a brain biopsy, the combination of focal neurological symptoms, abnormal autoantibodies and positive response to immunotherapy strongly indicated a diagnosis of NPSLE. It is necessary to continue to follow up the patient in case of complications with other systemic injuries.

Both central (CNS) and peripheral (PNS) nervous systems may be involved in SLE in addition to several psychiatric manifestations, although the diagnosis can be difficult [8]. Currently, the diagnosis of SLE remains a clinical diagnosis [9]. 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]. 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.

The pathogenesis of neuropsychiatric events in lupus patients remains unclear, making diagnosis and treatment challenging. Complex mechanisms such as blood-brain barrier disruption, vascular occlusion, neuroendocrine immune imbalance, tissue and neuronal damage mediated by autoantibodies and inflammatory mediators, and direct neuronal cell death may be involved [11]. Focal neurological symptoms are more common in NPSLE patients because SLE is more prone to endothelial dysfunction caused by autoimmune or ischemic complications, including vasculitis and thrombosis [12]. For active neuropsychiatric disease caused by SLE, it is recommended to consider glucocorticoids and immunosuppressants to treat inflammatory manifestations. In addition, the use of antiplatelet agents/anticoagulants is recommended for atherothrombosis/ antiphospholipid antibody-related manifestations [9]. For patients who achieve long-term remission, consider tapering the corticosteroid dose. There is no doubt that SLE requires multi-disciplinary, individualized management, including patient education and shared decision-making, taking into account costs to the patient and society [9].

Conclusions

It is uncommon for SLE to present with intracranial mass lesions as the initial presentation. When mass lesions of the brain occur rapidly in young adults, inflammatory lesions such as SLE should also be taken into account in addition to considering tumors, especially metastatic tumors. The pathogenesis of neurological symptoms in SLE patients may involve thrombosis or vasculitis leading to cerebral necrosis. Early initiation of immunotherapy and personalized management can improve prognosis in these cases.

Figures

The abnormal recorded electroencephalograms (EEGs) at admission. During the patient’s first hospitalization, the EEG showed slow and sustained activity on the right side, as indicated by delta waves (A). During the second hospitalization, the EEG revealed constant low voltage in the anterior head leads, which was clearer in right side recording (B).Figure 1.. The abnormal recorded electroencephalograms (EEGs) at admission. During the patient’s first hospitalization, the EEG showed slow and sustained activity on the right side, as indicated by delta waves (A). During the second hospitalization, the EEG revealed constant low voltage in the anterior head leads, which was clearer in right side recording (B). Brain images obtained on admission. Magnetic resonance imaging (MRI) at initial presentation revealed multiple abnormal lesions in the right frontoparietal lobe (white arrows), characterized by low signal intensity on T1-weighted sequences (A) and high signal intensity on T2-weighted sequences (B) and fluid-attenuated inversion recovery (FLAIR) sequences (C). The exact nature of these lesions remained uncertain. No notable abnormalities were observed in the N-acetyl-aspartate (NAA) and choline (Cho) peaks, while an inverted lactate peak was detected at 1.33 ppm (D). A follow-up brain MRI performed 20 days later revealed a decrease in T1 signal (E), an increase in T2 signal (F), and a decrease in FLAIR signal (G) in the right frontal lobe lesion compared with the initial examination (A–C), indicating liquefaction and necrosis. Magnetic resonance spectroscopy (MRS) revealed reduced NAA peak and abnormal lactate peak (H). During a second attack over a month later, new abnormal signal shadows (white arrows) in the right parietal lobe and left frontotemporal and parieto-occipital lobes in comparison with the initial hospitalization were obvious (I–K). The adjacent brain tissue was compressed, causing a shift of midline structures to the right (black arrow). The observed lesions exhibited characteristics consistent with vascular distribution. Subsequent analysis of computed tomography angiography and computed tomography venography did not reveal any significant abnormalities in the major blood vessels (L, P). After 15 days of treatment, a follow-up MRI showed the smaller focus with signs of necrolysis and liquefaction (M–O), prompting consideration of thrombosis or inflammation in small blood vessels leading to tissue necrosis.Figure 2.. Brain images obtained on admission. Magnetic resonance imaging (MRI) at initial presentation revealed multiple abnormal lesions in the right frontoparietal lobe (white arrows), characterized by low signal intensity on T1-weighted sequences (A) and high signal intensity on T2-weighted sequences (B) and fluid-attenuated inversion recovery (FLAIR) sequences (C). The exact nature of these lesions remained uncertain. No notable abnormalities were observed in the N-acetyl-aspartate (NAA) and choline (Cho) peaks, while an inverted lactate peak was detected at 1.33 ppm (D). A follow-up brain MRI performed 20 days later revealed a decrease in T1 signal (E), an increase in T2 signal (F), and a decrease in FLAIR signal (G) in the right frontal lobe lesion compared with the initial examination (A–C), indicating liquefaction and necrosis. Magnetic resonance spectroscopy (MRS) revealed reduced NAA peak and abnormal lactate peak (H). During a second attack over a month later, new abnormal signal shadows (white arrows) in the right parietal lobe and left frontotemporal and parieto-occipital lobes in comparison with the initial hospitalization were obvious (I–K). The adjacent brain tissue was compressed, causing a shift of midline structures to the right (black arrow). The observed lesions exhibited characteristics consistent with vascular distribution. Subsequent analysis of computed tomography angiography and computed tomography venography did not reveal any significant abnormalities in the major blood vessels (L, P). After 15 days of treatment, a follow-up MRI showed the smaller focus with signs of necrolysis and liquefaction (M–O), prompting consideration of thrombosis or inflammation in small blood vessels leading to tissue necrosis.

References:

1.. Unterman A, Nolte JE, Boaz M, Neuropsychiatric syndromes in systemic lupus erythematosus: A meta-analysis, 2011; 41(1); 1-11

2.. Obermoser G, Sontheimer RD, Zelger B, Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates: Lupus, 2010; 19(9); 1050-70

3.. Hanly JG, Urowitz MB, Sanchez-Guerrero J, Neuropsychiatric events at the time of diagnosis of systemic lupus erythematosus: An international inception cohort study: Arthritis Rheum, 2007; 56(1); 265-73

4.. Steup-Beekman GM, Zirkzee EJ, Cohen D, Neuropsychiatric manifestations in patients with systemic lupus erythematosus: Epidemiologyand radiology pointing to an immune-mediated cause: Ann Rheum Dis, 2013; 72(Suppl. 2); ii76-79

5.. Justiz Vaillant AA, Goyal A, Varacallo M, Systemic lupus erythematosus: StatPearls August 4, 2023, Treasure Island (FL), StatPearls Publishing https://pubmed.ncbi.nlm.nih.gov/30571026/

6.. Aringer M, Costenbader K, Johnson SR, Assessing the EULAR/ACR classification criteria for patients with systemic lupus erythematosus: Expert Rev Clin Immunol, 2022; 18(2); 135-44

7.. Gladman DD, Ibañez D, Urowitz MB, Systemic lupus erythematosus disease activity index 2000: J Rheumatol, 2002; 29(2); 288-91

8.. Hanly JG, Inanç M, The neurology of lupus: J Neurol Sci, 2021; 424; 117419

9.. Fanouriakis A, Kostopoulou M, Andersen J, EULAR recommendations for the management of systemic lupus erythematosus: 2023 update: Ann Rheum Dis, 2024; 83(1); 15-29

10.. Kim NR, Kang JW, Nam EJ, Tumor-like presentation of cerebral vasculitis in a patient with systemic lupus erythematosus: A biopsy-confirmed case: J Rheum Dis, 2023; 30(1); 53-57

11.. Jeltsch-David H, Muller S, Neuropsychiatric systemic lupus erythematosus: Pathogenesis and biomarkers: Nat Rev Neurol, 2014; 10(10); 579-96

12.. Hanly JG, Kozora E, Beyea SD, Birnbaum J, Review: Nervous system disease in systemic lupus erythematosus: Current status and future directions: Arthritis Rheumatol, 2019; 71(1); 33-42

Figures

Figure 1.. The abnormal recorded electroencephalograms (EEGs) at admission. During the patient’s first hospitalization, the EEG showed slow and sustained activity on the right side, as indicated by delta waves (A). During the second hospitalization, the EEG revealed constant low voltage in the anterior head leads, which was clearer in right side recording (B).Figure 2.. Brain images obtained on admission. Magnetic resonance imaging (MRI) at initial presentation revealed multiple abnormal lesions in the right frontoparietal lobe (white arrows), characterized by low signal intensity on T1-weighted sequences (A) and high signal intensity on T2-weighted sequences (B) and fluid-attenuated inversion recovery (FLAIR) sequences (C). The exact nature of these lesions remained uncertain. No notable abnormalities were observed in the N-acetyl-aspartate (NAA) and choline (Cho) peaks, while an inverted lactate peak was detected at 1.33 ppm (D). A follow-up brain MRI performed 20 days later revealed a decrease in T1 signal (E), an increase in T2 signal (F), and a decrease in FLAIR signal (G) in the right frontal lobe lesion compared with the initial examination (A–C), indicating liquefaction and necrosis. Magnetic resonance spectroscopy (MRS) revealed reduced NAA peak and abnormal lactate peak (H). During a second attack over a month later, new abnormal signal shadows (white arrows) in the right parietal lobe and left frontotemporal and parieto-occipital lobes in comparison with the initial hospitalization were obvious (I–K). The adjacent brain tissue was compressed, causing a shift of midline structures to the right (black arrow). The observed lesions exhibited characteristics consistent with vascular distribution. Subsequent analysis of computed tomography angiography and computed tomography venography did not reveal any significant abnormalities in the major blood vessels (L, P). After 15 days of treatment, a follow-up MRI showed the smaller focus with signs of necrolysis and liquefaction (M–O), prompting consideration of thrombosis or inflammation in small blood vessels leading to tissue necrosis.

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923