What are the diagnostic criteria for Neuropsychiatric Systemic Lupus Erythematosus (SLE) cerebritis?

From the Guidelines

Lupus cerebritis is diagnosed through a comprehensive approach that includes clinical evaluation, laboratory tests, and neuroimaging, with MRI being the preferred imaging method, as recommended by the EULAR task force 1. The diagnosis of lupus cerebritis involves a combination of clinical findings, laboratory tests, and imaging results.

• Clinical evaluation: Doctors look for neuropsychiatric symptoms like headaches, seizures, confusion, or mood changes in patients with systemic lupus erythematosus (SLE).
• Laboratory tests: Blood tests are essential, including complete blood count, anti-nuclear antibody (ANA), anti-double-stranded DNA antibodies, and complement levels (C3, C4).
• Neuroimaging: MRI is the preferred imaging method, which may reveal white matter lesions, infarcts, or edema. The American College of Rheumatology criteria for neuropsychiatric lupus helps guide diagnosis, and early diagnosis is crucial as prompt treatment with corticosteroids (often starting with prednisone 1-2 mg/kg/day), immunosuppressants like cyclophosphamide or mycophenolate mofetil, and sometimes anticoagulants can significantly improve outcomes and prevent permanent neurological damage 1. Some key points to consider in the diagnosis and management of lupus cerebritis include:
• The use of advanced MRI techniques and/or functional neuroimaging in cases of normal MRI readings or when MRI findings do not correlate with the clinical syndrome.
• The importance of excluding other causes of neurological symptoms, such as infection or stroke.
• The need for prompt treatment with immunosuppressive therapy, such as glucocorticoids and cyclophosphamide, in severe cases of lupus cerebritis.
• The potential benefits of anticoagulation therapy in patients with antiphospholipid antibodies and thrombotic events.

From the Research

Diagnosis of Lupus Cerebritis

• Lupus cerebritis is a manifestation of systemic lupus erythematosus (SLE) that affects the central nervous system, occurring in 24%-50% of SLE patients in the United States at some time during the course of their illness 2.
• The diagnosis of lupus cerebritis can be challenging due to its nonspecific symptoms, such as headache, seizures, stroke, and chorea 2.
• Advances in imaging and laboratory analysis have contributed to an earlier and more specific diagnosis of lupus cerebritis 2.

Clinical Features

• The main symptoms of CNS lupus can be diffuse (generalized seizures, psychosis) or focal (stroke, peripheral neuropathies) 3.
• Neuropsychiatric symptoms often occur in the first year of SLE, but are rarely the presenting symptoms of the disease 3.
• A case study reported a 23-year-old female with no significant past medical history, presenting with nausea and vomiting for two weeks and unusual behavior for three days, who was diagnosed with SLE and lupus cerebritis 4.
• Another case study reported a 31-year-old female presenting with psychosis, who was diagnosed with lupus cerebritis as the initial presentation of SLE 5.

Imaging Features

• Magnetic resonance imaging (MRI) scans often provide evidence for edema or small infarcts, both in focal and diffuse CNS lupus, whereas computerized tomography (CT) scans only show gross abnormalities 3.
• Position emission tomography (PET) scans in CNS lupus patients show decreased glucose uptake in the brain 3.
• A study reported that the cerebral blood flow decreases during active diffuse and focal CNS lupus 3.
• Radiologists and clinicians should become familiar with the underlying mechanisms, radiologic findings, and complications of NPSLE, as this information may aid in the diagnosis and treatment of NPSLE 6.

Pathophysiology

• The pathophysiology of NPSLE is complex and multifactorial, involving various immune effectors and the brain-intrinsic neuroimmune interfaces that are breached by the immune effectors 6.
• The underlying pathophysiology can be classified as vasculitis and vasculopathy, APS, demyelinating syndrome, or autoimmune antibody-mediated encephalitis, each with different imaging characteristics 6.
• Anticardiolipin (ACA) antibodies show a well-documented association with focal involvement of the CNS in SLE, and may cause thrombosis by interfering with the protein C pathway of fibrinolysis 3.