In order to better understand the clinical manifestation of systemic lupus erythematosus (SLE) with intracranial hypertension syndrome (IHS), we analyzed the clinical features and treatment of a typical SLE patient with IHS. SLE is one of the most unpredictable autoimmune diseases involving multiple organ systems that is defined clinically and associated with antibodies directed against cell nuclei. IHS is an uncommon manifestation of neuropsychiatric SLE (NPSLE) and is characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. IHS has been reported in a few sporadic cases in patients with SLE worldwide, but rarely has been reported in China. In this study, a 34-year-old female SLE patient with IHS was reported and pertinent literature reviewed. The clinical presentation, image logical features, and investigatory findings were discussed.
Diagnosis, Differential ; Intracranial Hypertension/diagnosis ; Intracranial Hypertension/*etiology ; Lupus Erythematosus, Systemic/*complications ; Lupus Erythematosus, Systemic/diagnosis

Child ; Female ; Hemoptysis ; etiology ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis

T-cell DNA hypomethylation can increase expression of genes that have potential relation to autoimmunity. CD70 overexpression may be associated with B-cell activation and immunoglobulin secretion, while perforin plays an important role in T-cell-mediated macrophage apoptosis. DNA hypomethylation can activate human endogenous retroviruses (HERV) sequences. Expression of HERV components may elicit autoantibodies production. Polyamines change structure of chromosome and interfere with DNA methylation process, which is involved in the pathogenesis of autoimmune diseases.
DNA Methylation ; Humans ; Lupus Erythematosus, Systemic ; etiology ; genetics ; immunology

Child ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; Myxoma ; etiology

Atherosclerosis ; etiology ; therapy ; Humans ; Lupus Erythematosus, Systemic ; pathology ; therapy

Anemia, Hemolytic ; etiology ; Humans ; Lupus Erythematosus, Systemic ; complications ; Syndrome

Adolescent ; Female ; Humans ; Hypothyroidism ; etiology ; Lupus Erythematosus, Systemic ; complications

Neutrophil extracellular traps (NETs) represent a form of cell death distinct from apoptosis or necrosis. The imbalance between the formation and degradation of NETs has long been considered to be closely associated with the activity of autoimmune diseases such as systemic lupus erythematous (SLE). Reactive oxygen species derived from the nicotinamide adenine dinucleotide phosphate oxidase pathway or mitochondrial DNA pathway play a key role in the primary stage of NETs formation. The exposure or delayed degradation of abundant autoantigens, such as double-strand DNA, caused by abnormal activation of neutrophils can induce autoantibody to form immune complexes that deposit in local tissues and then induce the plasmacytoid dendritic cells to secrete the interferon alpha and other inflammatory factors. Those inflammatory factors will eventually cause endothelial cell injury. In order to provide a theoretical basis for targeted therapy and diagnosis of childhood-onset SLE, this paper reviews the role of NETs in the pathogenesis of SLE.
Extracellular Traps ; Humans ; Lupus Erythematosus, Systemic ; etiology ; therapy ; Neutrophils ; physiology

Chorea/etiology* ; Humans ; Lupus Erythematosus, Systemic/diagnosis* ; Phototherapy

Humans ; Hearing Loss/etiology* ; Lupus Erythematosus, Systemic/complications* ; Prognosis