Introduction and state of the art: Systemic lupus erythematosus (SLE) is an autoimmune disease that affects many organs throughout its course, most frequently the joints, skin and kidneys. Both the central (CNS) and peripheral (PNS) nervous systems are also often affected. T he involvement of the CNS has a negative prognosis in lupus patients. Neurological symptoms are diverse, from headaches and cognitive dysfunction to life-threatening seizures or stroke. Due to the great diversity of neurological presentations, diagnosing neuropsychiatric SLE (NPSLE, neurolupus) can be challenging and necessitates a careful differential diagnostic work-up. Furthermore, neurological symptoms can be one of the first signs of the disease, making the correct diagnosis even more challenging. White matter lesions in NPSLE may closely resemble lesions formed during multiple sclerosis (MS), which is a chronic autoimmune disease of the CNS resulting in neuroinflammatory damage to the myelin sheath, axonal impairment, and neurodegeneration. Based on imaging only, it is challenging to differentiate between the two diseases.
Clinical implications: While both diseases have characteristic features, in their early stages they may mimic each other. The purpose of this literature review was to emphasise the differences in clinical, immunological and neuroimaging features between the two diseases in order to facilitate diagnosis, highlighting the most useful diagnostic tools.
Future directions: Prompt and accurate diagnosis is crucial for implementing appropriate, disease-specific treatment and thereby improving the prognosis for the patient. Therefore, there is a need for novel imaging and laboratory biomarkers, possibly used as a multifactorial profile, to differentiate NPSLE from MS.
Keywords: CSF; MRI; autoantibodies; cerebrospinal fluid; multiple sclerosis; neurolupus; neuropsychiatric SLE; systemic lupus erythematosus.