Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus (jSLE)

Abstract

Systemic lupus erythematosus (SLE) is a rare autoimmune/inflammatory disease with significant morbidity and mortality. Approximately 15–20% of SLE patients develop the disease during childhood or adolescence (juvenile-onset SLE/jSLE). Patients with jSLE exhibit more variable and severe disease when compared to patients with disease-onset during adulthood. Neuropsychiatric (NP) involvement is a clinically heterogenous and potentially severe complication. Published reports on the incidence and prevalence of NP-jSLE are scarce, and the exact pathophysiology is poorly understood.This manuscript provides a review of the existing literature, suggesting NP involvement in 13.5–51% of jSLE patients. Among patients with NP-jSLE affecting the CNS, we propose two main subgroups: (i) a chronic progressive, predominantly type 1 interferon-driven form that poorly responds to currently used treatments, and (ii) an acutely aggressive form that usually presents early during the disease that may be primarily mediated by auto-reactive effector lymphocytes. While this hypothesis requires to be tested in large collaborative international cohort studies, it may offer future patient stratification and individualised care.

Magnetic resonance imaging of the musculoskeletal system in the diagnosis of rheumatic diseases in the pediatric population

“…Typical MRI changes in JSLE include the presence of edematous tendinitis and edema of the joint capsule [ 74 ]. In addition, brain MRI remains a significant feature in JSLE, and is performed in the case of coexisting neurological symptoms even though there are no specific neuro-radiological findings that are definitive for neuropsychiatric JSLE, and imaging can be normal, even in cases with small vessel central nervous system vasculitis [ 75 ]. Magnetic resonance imaging in children indicates that white matter hyperintensity is the most commonly observed lesion in patients with abnormal MRI findings [ 76 ].…”

Immune-mediated inflammatory diseases (IMIDs) in children: key research questions and some answers

“…pSLE is characterized by a multifaceted molecular pathophysiology and manifests with multiple phenotypes. The work by Valentina Natoli et al is dedicated to a rare and poorly described presentation of pSLE [13]. Their comprehensive review proposes two subgroups of neuropsychiatric SLE: (i) a chronic progressive, predominantly type 1 interferon-driven form that poorly responds to currently used treatments, and (ii) an acutely aggressive form that usually presents early during the disease that may be primarily mediated by auto-reactive effector lymphocytes.…”