Recommendations for NP-cSLE—diagnostic procedures and treatment
| Level of evidence | Strength | Agreement (%) | |
|---|---|---|---|
| Diagnostic recommendations | |||
| 1. The nomenclature and case definitions for NP-cSLE syndromes proposed by the ACR ad hoc committee should be used to classify and describe NP-SLE syndromes in cSLE. | 3 | C | 100 |
| 2. In patients with cSLE with new or unexplained symptoms or signs suggestive of neuropsychiatric disease, initial diagnostic work-up should include work-up as performed in patients without SLE. | 3 | C | 100 |
| 3. In a patient with a suspected diagnosis of NP-cSLE or worsening NP-cSLE symptoms, underlying factors including infections, hypertension, metabolic abnormalities or adverse effects of medication should be excluded. | 3 | C | 100 |
| 4. Depending upon the type of neuropsychiatric manifestation, the diagnostic work-up may include lumbar puncture and CSF analysis (primarily to exclude CNS infection), EEG, neuropsychological assessment of cognitive function, consultation with an ophthalmologist, nerve conductional studies and neuroimaging (MRI) to assess nervous system structure and function.* | 3 | C | 100 |
| 5. A normal MRI of the CNS does not exclude NP-cSLE.* | 3 | C | 100 |
| 6. Cognitive impairment should be tested either in collaboration with a neuropsychologist, or using validated tests for cognitive impairment in cSLE, like the Ped-ANAM. | 3 | C | 100 |
| Treatment recommendations | |||
| 1. When neuropsychiatric manifestations are caused by an immune or inflammatory process and non-SLE-related causes are excluded, corticosteroids and immunosuppressive therapy are indicated. | 3 | C | 100 |
| 2. Antiepileptic drugs are usually not necessary after a single seizure in the absence of MRI lesions and definite epileptic abnormalities on EEG following recovery from the seizure.* | 3 | C | 100 |
| 3. Long-term antiepileptic therapy should be considered for recurrent seizures.* | 3 | C | 93 |
| 4. There is a need for paediatric NP-cSLE research regarding treatment. | 4 | D | 100 |
↵*This statement is based on the EULAR recommendations for adults with NP-cSLE.68 123
Level of evidence: for diagnostic and observational studies: 1A, meta-analysis of cohort studies; 1B, randomised controlled study; 2A, controlled study without randomisation; 2B, quasiexperimental study; and for treatment studies: 1A, meta-analysis of randomised controlled trial; B, based on level 2 or extrapolated from level 1; C, based on level 3 or extrapolated from level 1 or 2; D, based on level 4 or extrapolated from level 3 or 4 expert opinion.22 Strength of recommendation: A, based on level 1 evidence; 3, descriptive study; 4, expert opinion.25–27 Agreement indicates percent of experts agreeing on the recommendation during the final voting round of the consensus meeting.
ACR, American College of Rheumatology; CNS, central nervous system; CSF, cerebrospinal fluid; EEG, electroencephalogram; EULAR, European League Against Rheumatism; NP-cSLE, neuropsychiatric childhood-onset systemic lupus erythematosus; NP-SLE, neuropsychiatric systemic lupus erythematosus; Ped-ANAM, Pediatric Automated Neuropsychological Assessment Metrics; SLE, systemic lupus erythematosus.