scholarly journals Differences between central nervous system infection and neuropsychiatric systemic lupus erythematosus in patients with systemic lupus erythematosus

2017 ◽  
Vol 46 (1) ◽  
pp. 485-491 ◽  
Author(s):  
Hongli Fang ◽  
Likang Lan ◽  
Yanzhou Qu ◽  
Qiankun Zhang ◽  
Jin Lv

Objective Neuropsychiatric systemic lupus erythematosus (NPSLE) is a manifestation of systemic lupus erythematosus (SLE). Central nervous system (CNS) infection is a consequence of intensive immunosuppressive therapy that patients with SLE might undergo. This study aimed to compare the differences between NPSLE and CNS infections in patients with SLE. Methods Patients with SLE and NPSLE or CNS infections were retrospectively reviewed. Clinical manifestations, laboratory test results, and prognoses were recorded. The independent sample t-test or chi-square test was used to compare data. Results Patients with CNS infections (n = 20) had more serious headache, high fever (>39.0°C), and vomiting compared with patients with NPSLE (n = 48). Patients with CNS infections also had a larger prednisone dose at the time of symptom onset, larger cumulative dosages over the preceding year, lower SLE Disease Activity Index (SLEDAI) scores, higher rate of nephritis, lower albumin levels, higher C-reactive protein (CRP) levels, higher 24-h-urine protein levels, higher cerebrospinal fluid (CSF) white blood cell levels, and lower protein and glucose levels than those with NPSLE. Conclusions For patients with SLE presenting with CNS symptoms, serious headache, high fever, a high dose of corticosteroids, low SLEDAI scores, and abnormal CSF are more important indicators for CNS infections than NPSLE.

Author(s):  
Elias Manca

AbstractSystemic lupus erythematosus is a complex immunological disease where both environmental factors and genetic predisposition lead to the dysregulation of important immune mechanisms. Eventually, the combination of these factors leads to the production of self-reactive antibodies that can target any organ or tissue of the human body. Autoantibodies can form immune complexes responsible for both the organ damage and the most severe complications. Involvement of the central nervous system defines a subcategory of the disease, generally known with the denomination of neuropsychiatric systemic lupus erythematosus. Neuropsychiatric symptoms can range from relatively mild manifestations, such as headache, to more severe complications, such as psychosis. The evaluation of the presence of the autoantibodies in the serum of these patients is the most helpful diagnostic tool for the assessment of the disease. The scientific progresses achieved in the last decades helped researchers and physicians to discover some of autoepitopes targeted by the autoantibodies, although the majority of them have not been identified yet. Additionally, the central nervous system is full of epitopes that cannot be found elsewhere in the human body, for this reason, autoantibodies that selectively target these epitopes might be used for the differential diagnosis between patients with and without the neuropsychiatric symptoms. In this review, the most relevant data is reported with regard to mechanisms implicated in the production of autoantibodies and the most important autoantibodies found among patients with systemic lupus erythematosus with and without the neuropsychiatric manifestations.


Author(s):  
Bonnie L. Bermas

Systemic lupus erythematosus (SLE) is a multisystem disease that preferentially affects women of childbearing age. This disorder is both more common and more severe in individuals of African and Asian ancestry. The etiology of SLE is not well understood, although genetics and environmental stimuli clearly are involved. Whether this disease is caused by a T-cell, B-cell, or other immunologic malfunction is debated, but all would agree that clearly autoantibodies such as antinuclear antibodies and anti–double-stranded DNA contribute to the pathophysiology of this disorder. This multisystem disease can affect the skin, joints, lungs, heart, kidneys, and central nervous system. Most of the morbidity and mortality is from renal and central nervous system (CNS) involvement, although accelerated atherosclerosis has recently been appreciated as a major contributor to disease burden. The treatment of SLE has improved over the past decade with less reliance on high-dose corticosteroids and more emphasis on immunosuppressive agents. It is our hope that future research into the pathophysiology of this disorder and the development of more specific therapy, such as biologics, will improve the outcome of this disease.


Author(s):  
Toru Sakairi ◽  
Masao Nakasatomi ◽  
Mitsuharu Watanabe ◽  
Hiroko Hamatani ◽  
Hidekazu Ikeuchi ◽  
...  

ABSTRACT A 41-year-old woman with a 14-month history of systemic lupus erythematosus (SLE) presented with headache, aphasia, and agraphia. A laboratory examination revealed mild proteinuria, hypocomplementemia, and elevated anti-double-stranded DNA antibody levels. A cerebrospinal fluid analysis demonstrated elevated protein and interleukin-6 levels. Magnetic resonance imaging (MRI) of the brain identified multiple lesions suggestive of brain edemas and small haemorrhages. She was diagnosed as having neuropsychiatric lupus and lupus nephritis and received remission induction therapy with high-dose corticosteroid and intravenous cyclophosphamide. She achieved a complete remission, and treatment with mycophenolate mofetil (MMF) was initiated 3 months thereafter for remission maintenance. At 13 months after the exacerbation of SLE, she complained of headache and nausea. A gadolinium-enhanced MRI of the brain revealed a low-signal-intensity tumour with marginal ring enhancement of 50 mm in the left frontal lobe. The tumour was excised, and the histological diagnosis was diffuse large B-cell lymphoma with positive Epstein–Barr virus (EBV). MMF was discontinued. Remission induction therapy with rituximab, high-dose methotrexate, procarbazine, and vincristine was administered, and she achieved remission. Previous reports suggest that use of MMF is associated with primary central nervous system (CNS) lymphoma (PCNSL) in patients with lupus nephritis or other autoimmune diseases or in post-transplant patients. Our observation that PCNSL occurred after CNS involvement of SLE suggests that EBV and CNS inflammation arising from SLE might have contributed to the development of PCNSL.


Lupus ◽  
2017 ◽  
Vol 26 (5) ◽  
pp. 490-496 ◽  
Author(s):  
F M Ribeiro ◽  
F Signorelli

Opportunistic infections can cause manifestations that resemble neuropsychiatric systemic lupus erythematosus and they can also trigger lupus flares. Therefore, central nervous system infections as differential diagnosis in neuropsychiatric systemic lupus erythematosus may be difficult, leading to delayed diagnosis and specific treatment. Central nervous system infection in systemic lupus erythematosus is not common but, if left misdiagnosed and not treated promptly, can be fatal. Complementary diagnosis tests are generally non-specific and disappointing. Caution with immunosuppressive drug treatment should be emphasized while an opportunistic infection cannot be ruled out. In this review, we discuss the various types of central nervous system infections reported in systemic lupus erythematosus patients, highlighting the importance of their early recognition in order to improve morbidity and mortality. Prevention with vaccination is a recommended approach.


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