Table 2.

Definition and diagnostic criteria for neuropsychiatric lupus erythematosus.

Definition of NPSLE NPSLE is a group of neurologic syndromes of the central and peripheral nervous system and/or of psychiatric syndromes observed in patients with SLE in which other causes are excluded by laboratory, clinical, neuropsychological tests and neuroimaging.

Prerequisite for diagnosis: All the author’s criteria for NPSLE require the diagnosis of SLE. Symptoms and signs: 1. Altered cognitive and/or neuropsychiatric status manifestations or sensorimotor neuropathy. 2. Higher SLEDAI score (>10). 3. Antiphospholipid (APL), anti-ribosomal-P (ARP), anti-neuronal, anti-ganglioside (GMI) and anti-Ro antibody, one of the antibodies is positive. 4. Complicated with tissue infarction, hemorrhage, or more limited focal neuron injury results from impaired blood low (thrombosis). 5. Skin lesions

Diagnosis Grade of NPSLE combinations of features requirements for diagnosis NPSLE1: SLE plus at least one altered cognitive and/or neuropsychiatric status manifestations or sensorimotor neuropathy and one of the following: higher SLEDAI, antibody positivity, thromboembolism, or skin lesions. NPSLE2: SLE and at least one altered cognitive and/or neuropsychiatric status manifestations or sensorimotor neuropathy.

Exclusion and provisions Secondary causes such as medication side effects (especially steroids), thyroid disease, infections, metabolic disturbances, TTP, valvular heart disease, depression, sleep apnea, and psychosocial- or functional-related conditions need to be excluded.

NPSLE 1: ‘‘definite’’ NPSLE; NPSLE 2: ‘‘suspected’’ NPSLE. TTP: thrombotic thrombocytopenic purpura.