| Domain | Finding (with numbers) | Population/Design | Source (first author, year, journal) | Publication date | URL | Notes |
|---|---|---|---|---|---|---|
| Epidemiology | Reported NPSLE prevalence in literature ranges **12–95%** | Review of diagnostic approaches and literature heterogeneity | Emerson, 2023, *Frontiers in Neurology* | 2023-03-21 | https://doi.org/10.3389/fneur.2023.1111769 | Wide range reflects inconsistent definitions, study design, and populations; supports need for composite diagnostic panels (pqac-00000005, pqac-00000016) |
| Epidemiology | Across cohorts using ACR nomenclature, overall NP prevalence **37–95%**; common syndromes: cognitive dysfunction **55–80%**, headache **24–72%**, mood disorder **14–57%**, cerebrovascular disease **5–18%**, seizures **6–51%**, polyneuropathy **3–28%**, anxiety **7–24%**, psychosis **0–8%** | Review of representative cohort studies using ACR 1999 NP-SLE criteria | Hanly, 2005, *Best Practice & Research Clinical Rheumatology* | 2005-10 | https://doi.org/10.1016/j.berh.2005.04.003 | Also notes up to **41%** of NP events may be attributable to non-lupus causes, underscoring diagnostic exclusion (pqac-00000007, pqac-00000017) |
| Epidemiology | In systematic review, status epilepticus and transverse myelitis each **1–2%**; cognitive dysfunction nearly **38%** | Systematic review of 11 studies | Rice-Canetto, 2024, *Cureus* | 2024-06 | https://doi.org/10.7759/cureus.61678 | Prognosis varies by syndrome; diagnostics often include brain MRI and antibody testing (pqac-00000000) |
| Biomarkers | Healthy controls: mean blood **NfL 3.6 pg/mL (SD 2.0)** and **GFAP 50.4 pg/mL (SD 15.0)**; active major NPSLE vs SLE controls: **NfL +17.9 pg/mL** (95% CI 9.2–34.5, **p<0.001**) and **GFAP +3.2 pg/mL** (95% CI 1.9–5.5, **p<0.001**); SLE controls vs healthy controls: NfL **+1.3 pg/mL** (p=0.42), GFAP **+1.2 pg/mL** (p=0.53) | Case-control study; **13 active major NPSLE**, **13 SLE controls**, **13 healthy controls**; mean ages 26.8/27.3/26.6 years; 92% female | Kammeyer, 2024, *Lupus* | 2024-08 | https://doi.org/10.1177/09612033241272961 | Biomarkers decreased after immunotherapy in a subset; blood-CSF correlations: NfL **r=0.88, p=0.01**, GFAP **r=0.81, p=0.03** (pqac-00000012, pqac-00000013) |
| Biomarkers | Among **51** j-SLE patients, **39%** had j-NPSLE; j-NPSLE diagnosed at SLE onset in **65%**; CSF neopterin higher in active j-NPSLE with CNS involvement vs j-SLE alone (**p=0.0008**); CSF neopterin and IFN-α decreased after resolution (**p=0.0015** and **p=0.0010**); biomarkers strongly correlated (**Rs=0.832, p<0.0001, n=23 paired samples**) | 5-year retrospective monocentric pediatric cohort | Labouret, 2023, *Journal of Clinical Immunology* | 2023-12 | https://doi.org/10.1007/s10875-022-01407-1 | No specific routine biological or radiological marker identified; supports CSF IFN-α/neopterin as promising activity biomarkers (pqac-00000014, pqac-00000015) |
| Treatment outcomes | At **12 months**, **224/350 (64%)** improved clinically; focal central events improved in **66/79 (83%)**; SLE-attributed events improved in **113/155 (72.9%)** by algorithm and about **73.0%** by clinical judgment; immunosuppressant-treated, clinically judged SLE-attributed NP events had higher response odds: **OR 2.55** (95% CI 1.06–6.41, **p=0.04**) | International multicenter retrospective cohort of first NP event; **350 events** analyzed from **362** with follow-up | Bortoluzzi, 2024, *Rheumatology* | 2024-02 | https://doi.org/10.1093/rheumatology/keae119 | Initial/escalated immunosuppressants and corticosteroids were used more often for central diffuse/focal SLE-attributed events; one-year outcomes support timely attribution and immunosuppression (pqac-00000010, pqac-00000011) |
| Guidelines | **HCQ recommended for all patients** at target dose **5 mg/kg real body weight/day**; maintenance glucocorticoids should be minimized to **≤5 mg/day prednisone equivalent** and withdrawn when possible; CYC for organ-threatening disease and rituximab for refractory disease | International EULAR guideline update for SLE management | Fanouriakis, 2024, *Annals of the Rheumatic Diseases* | 2024-01 | https://doi.org/10.1136/ard-2023-224762 | General SLE guidance relevant to NPSLE management; emphasizes early steroid-sparing immunosuppression/biologics in appropriate patients (pqac-00000008) |
| Guidelines | For severe neuropsychiatric disease, **anifrolumab and belimumab are not recommended** | Figure-based treatment framework for non-renal SLE in EULAR 2023 update | Fanouriakis, 2024, *Annals of the Rheumatic Diseases* | 2024-01 | https://doi.org/10.1136/ard-2023-224762 | Figure 1 explicitly notes biologics are not recommended in **severe neuropsychiatric disease** despite broader extrarenal SLE roles (pqac-00000009, pqac-00000018) |


*Table: This table compiles high-yield quantitative findings and actionable guidance for neuropsychiatric systemic lupus erythematosus from the gathered evidence. It is useful as a quick reference for prevalence, biomarker performance, treatment outcomes, and current guideline implications.*