Autoimmune aquaporin-4 induced damage beyond the central nervous system

He, Dian; Zhang, Anni; Wang, Yongdong; Cai, Gang; Li, Yuan; Guo, Shipeng

doi:10.1016/j.msard.2017.09.013

Cited by 21 publications

(14 citation statements)

References 64 publications

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“…Aquaporin-4 (AQP4) is a water channel found on the foot processes of astrocyte cells in the brain and is also expressed in the renal tract, the gastrointestinal system, skeletal muscles, lung and blood system [1,2].…”

Section: Introductionmentioning

confidence: 99%

“…The discovery of pathogenic AQP4-Ab resulted in expansion of the clinical phenotypes associated with NMSOD to include patients with more atypical neuroinflammation even in the absence of optic neuritis and transverse myelitis but who test positive for these antibodies. Notably, in recent years, growing evidence suggests that AQP4-IgG may also causes damage to peripheral AQP4-expressing organs beyond the CNS such as skeletal muscle, vestibulocochlear nerves, gastrointestinal tract, blood system, kidney, lung and placenta [2]. So the spectrum of disease associated with these autoantibodies may expand over the years.…”

Section: Introductionmentioning

confidence: 99%

“…Notably, in recent years, growing evidence suggests that AQP4-IgG may also causes damage to peripheral AQP4-expressing organs beyond the CNS such as skeletal muscle, vestibulocochlear nerves, gastrointestinal tract, blood system, kidney, lung and placent. 2 So the spectrum of disease associated with these autoantibodies may expand over the years.…”

Section: Introductionmentioning

confidence: 99%

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Aquaporin-4 IgG antibody-related disorders in patients with juvenile systemic lupus erythematosus

Moraitis

Stathopoulos

Hong³

et al. 2019

Lupus

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Objective The aim of this study was to: (a) screen a large group of unselected patients with juvenile systemic lupus erythematosus for anti-aquaporin 4 antibodies (AQP4-Ab); (b) identify clinical and laboratory predictors of the presence of AQP4-Ab positivity in juvenile systemic lupus erythematosus. Methods Sera from 90 patients with juvenile systemic lupus erythematosus were tested for the presence of AQP4-Ab using a cell-based assay. Demographics, clinical and immunological features, treatment received were summarized. Fisher’s exact test was used to identify clinical predictors of positivity for AQP4-Ab. Results Five of 90 (5.5%) patients tested positive for AQP4-Ab, all of which had neurological involvement, mainly transverse myelitis and optic neuritis. AQP4-Ab-positive patients were more likely to have neurological symptoms ( P = 0.002), less likely to experience dermatological manifestations ( P = 0.045), and less likely to have detectable anti-dsDNA antibodies ( P = 0.022). These patients were also more likely to have received anti-epileptic ( P = 0.023) and anti-coagulant ( P = 0.007) drugs. Conclusions The findings of this study indicate that some patients with juvenile systemic lupus erythematosus develop antibodies against aquaporin-4 and may be at risk of developing a neurological clinical phenotype. We suggest that all juvenile systemic lupus erythematosus patients should be systematically screened for the presence of AQP4-Ab and this may help identify a high risk for neurological involvement in juvenile systemic lupus erythematosus.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Aquaporin-4 IgG antibody-related disorders in patients with juvenile systemic lupus erythematosus

Moraitis

Stathopoulos

Hong³

et al. 2019

Lupus

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“…The activation of the complement pathway induces a series of transcriptional events within affected astrocytes that further promotes granulocyte infiltration. The immune factors that are upregulated in reactive astrocytes include various cell adhesion molecules, oxidative stress‐related factors, inflammasome components, proteasome subunits, cytokines and chemokines . All these immune factors correlate with the immunopathological findings that eosinophils and neutrophils infiltrate NMOSD lesions (Figure ).…”

Section: The Binding Of the Aop4 Antigen And Antibody Within The Cnsmentioning

confidence: 99%

Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

Chang

2019

Neuropathology Appl Neurobio

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Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorderNeuromyelitis optica is an autoimmune inflammatory disorder of the central nervous system that preferentially targets the spinal cord and optic nerve. Following the discovery of circulating antibodies against the astrocytic aquaporin 4 (AQP4) water channel protein, recent studies have expanded our knowledge of the unique complexities of the pathogenesis of neuromyelitis optica and its relationship with the immune response. This review describes and summarizes the recent advances in our understanding of the molecular mechanisms underlying neuromyelitis optica disease pathology and examines their potential as therapeutic targets. Additionally, we update the most recent research by proposing major unanswered questions regarding how peripheral AQP4 antibodies are produced and their entry into the central nervous system, the causes of AQP4-IgG-seronegative disease, why peripheral AQP4-expressing organs are spared from damage, and the impact of this disease on pregnancy.

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“…Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune central nervous system disorder characterized by an unpredictable relapsing course and relapse-dependent cumulative disability (Huda et al, 2019;Mealy et al, 2019;Wingerchuk et al, 1999;Wingerchuk et al, 2007). The underlying immunopathogenic mechanism is autoimmunity against the aquaporin-4 (AQP4) water channel, resulting in complement-mediated astrocytic damage and subsequent neuronal injury (Duan et al, 2018;He et al, 2017;Hinson et al, 2012;Jiao et al, 2013;Saadoun et al, 2010). The safety and efficacy of eculizumab, a humanized monoclonal antibody that blocks the terminal complement system, were demonstrated in the pivotal phase 3 PREVENT trial (Pittock et al, 2019), in which eculizumab was well tolerated and the overall risk of adjudicated relapse was reduced by 94.2% versus placebo in adults with anti-AQP4 immunoglobulin G-positive ( Patients with NMOSD often have complex disease and treatment histories (Romeo and Segal, 2019).…”

Section: Introductionmentioning

confidence: 99%

Benefits of eculizumab in AQP4+ neuromyelitis optica spectrum disorder: Subgroup analyses of the randomized controlled phase 3 PREVENT trial

Palace

Wingerchuk

Fujihara

et al. 2021

Multiple Sclerosis and Related Disorders

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Autoimmune aquaporin-4 induced damage beyond the central nervous system

Cited by 21 publications

References 64 publications

Aquaporin-4 IgG antibody-related disorders in patients with juvenile systemic lupus erythematosus

Aquaporin-4 IgG antibody-related disorders in patients with juvenile systemic lupus erythematosus

Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

Benefits of eculizumab in AQP4+ neuromyelitis optica spectrum disorder: Subgroup analyses of the randomized controlled phase 3 PREVENT trial

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