• The study cohort included 111 primary SS and 43 non-SS Sjögren's International Collaborative Clinical Alliance (SICCA) controls that were obtained from the Sjögren's International Collaborative Clinical Alliance registry, in addition to 35 systemic lupus erythematosus (SLE) and 35 rheumatoid arthritis (RA) phenotypes. (mdpi.com)
  • Treatment of systemic lupus erythematosus (SLE) should be provided in cooperation with a consulting rheumatologist. (medscape.com)
  • The standard treatment for the nonthrombotic syndromes associated with systemic lupus erythematosus (SLE) is immunosuppression, first with corticosteroids and with early recourse to cyclophosphamide. (medscape.com)
  • The discovery that Toll-like receptor signaling and interferon-alpha abundance are central elements of the systemic lupus erythematosus (SLE) disease process has led to a new appreciation for hydroxychloroquine as an essential baseline medication. (medscape.com)
  • This is because antibodies to SSA and SSB are not specific to SjS, but are also found in other rheumatological diseases including systemic lupus erythematosus (SLE), and myositis [ 7 ]. (biomedcentral.com)
  • Aquaporin-4-autoimmunity in patients with systemic lupus erythematosus: A predominantly population-based study. (cdc.gov)
  • Certain neurological symptoms of neuromyelitis optica spectrum disorder (NMOSD) occur at higher rates in patients who also have systemic lupus erythematosus (SLE), according to a recent review study. (neuromyelitisnews.com)
  • The study, " Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and systemic lupus erythematosus: A systematic review of individual patient data ," was published in the journal Lupus . (neuromyelitisnews.com)
  • In contrast, secondary Sjogren's syndrome presents along with other autoimmune diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) [ rx] . (rxharun.com)
  • Systemic lupus erythematosus (SLE) is a complex connective tissue disorder that involves multiple organs. (bmj.com)
  • Background The presence of antibodies to aquaporin-4 (AQP4) has been identified as a key characteristic of neuromyelitis optica spectrum disorder (NMOSD), an autoimmune inflammatory demyelinating central nervous system (CNS) disorder. (guthyjacksonfoundation.org)
  • BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) stands out among CNS inflammatory demyelinating diseases (CIDDs) due to its unique disease characteristics, including severe clinical attacks with extensive lesions and its association with systemic autoimmune diseases. (bvsalud.org)
  • Most patients with neuromyelitis optica spectrum disorders (NMOSD) test positive for aquaporin-4 antibody (AQP4-IgG) or myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). (bvsalud.org)
  • For example, aquaporin-4 (AQP4) immunoglobulin G (IgG) serum autoantibody and myelin oligodendrocyte glycoprotein IgG (MOG IgG) autoantibody are specific for neuromyelitis optica spectrum disorders (NMOSDs) and MOG-associated disease (MOGAD), respectively. (arupconsult.com)
  • MOG antibody-associated disorder exhibits different pathophysiological and phenotypic findings than both aquaporin-4 antibody-associated neuromyelitis optica spectrum disorder and typical MS. MOG-antibody is of particular interest in pediatric patients with clinical or radiological non-MS typical findings. (e-cep.org)
  • Serum anti-neuromyelitis optica (NMO)/aquaporin-4 (AQP4) antibody was positive and was confirmed by repeat serology (cell-based assay, Mayo Clinic). (neurology.org)
  • Preliminary study on the association of AQP4 promoter polymorphism with anti-aquaporin-4 antibody positivity in southern Han Chinese patients with idiopathic demyelinating disorders of central nervous system. (cdc.gov)
  • NMOSD patients were positive for aquaporin-4 (AQP4) antibodies, the most common type of NMOSD-causing antibody. (neuromyelitisnews.com)
  • Of particular interest is the water channel aquaporin-4 (AQP4) which mislocalises in epilepsy. (dcu.ie)
  • Recently, autoimmune pathogenetic mechanisms related to neuromyelitis optica have been considered, based on the occurrence of specific NMO IgG antibodies against aquaporin 4. (csnn.eu)
  • The development of new immunosuppressant drugs and monoclonal antibodies has reduced the relapses and improved the prognosis of optic neuritis as well as an associated systemic disease. (lww.com)
  • METHODS: From a prospective cohort, we recruited patients with CIDDs and categorized them based on the presence and type of autoantibodies: NMOSD with anti-aquaporin-4 antibodies, myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) with anti-myelin oligodendrocyte glycoprotein antibodies, double-seronegative demyelinating disease (DSN), and healthy controls (HCs). (bvsalud.org)
  • Age was identified as a clinical factor associated with BCR activation specifically in NMOSD, implying the significance of persistent B cell activation attributed to anti-aquaporin-4 antibodies, even in the absence of clinical relapses throughout an individual's lifetime. (bvsalud.org)
  • Antibodies against the aquaporin 4 channel is an important evaluation for this common confusing situation. (medscape.com)
  • This extensive immune cross-reactivity between SARS-CoV-2 antibodies and different antigen groups may play a role in the multi-system disease process of COVID-19, influence the severity of the disease, precipitate the onset of autoimmunity in susceptible subgroups, and potentially exacerbate autoimmunity in subjects that have pre-existing autoimmune diseases. (frontiersin.org)
  • In this mechanism, antibodies formed against SARS-CoV-2 would also bind to human tissue proteins leading to autoimmune reactivity. (frontiersin.org)
  • Background: Neuromyelitis optica (NMO) is an inflammatory autoimmune disorder of the central nervous system, hallmarked by pathogenic anti-aquaporin 4 antibodies. (thestemcellfoundation.com)
  • Apart from other more common diseases (multiple sclerosis, inflammatory, neoplastic, systemic, vascular and metabolic causes of myelopathy), rare paraneoplastic spinal cord involvement must also be excluded. (csnn.eu)
  • 2 3 4 5 6 ] However, a more recent population-based survey conducted in urban Mangalore has shown a prevalence of 8.3/100,000 for MS and 2.6/100,000 for neuromyelitis optic spectrum diseases (NMOSD). (lww.com)
  • There is an increasing need for simple, sensitive and rapid technologies for the diagnosis of SjS and other autoimmune diseases. (biomedcentral.com)
  • One major approach involves using antibody-based tests to diagnose and even predict the onset of various diseases [ 1 , 4 ]. (biomedcentral.com)
  • Currently there are no serological tests for rapidly detecting autoantibodies associated with autoimmune diseases that also satisfy the growing demand for high analytical sensitivity and reproducibility. (biomedcentral.com)
  • Current systemic therapies are rarely curative for patients with severe life-threatening forms of autoimmune diseases (ADs). (thestemcellfoundation.com)
  • Association Between the Single Nucleotide Polymorphism and the Level of Aquaporin-4 Protein Expression in Han and Minority Chinese with Inflammatory Demyelinating Diseases of the Central Nervous System. (cdc.gov)
  • Features of NMOSD, including longitudinally extensive transverse myelitis - a type of spinal cord inflammation - and involvement of certain areas of the brain were seen at higher rates in patients with both autoimmune diseases than usually observed in NMOSD alone. (neuromyelitisnews.com)
  • Most patients were female (91.3%), consistent with the known female predominance in both autoimmune diseases. (neuromyelitisnews.com)
  • Primary Sjogren's syndrome (pSS) occurs in the absence of other autoimmune diseases and is characterized by keratoconjunctiva sicca (dry eyes) and xerostomia (dry mouth), collectively called the sicca syndrome. (rxharun.com)
  • Microfibrillar-associated protein 4 as a potential marker of acute relapse in inflammatory demyelinating diseases of the central nervous system: Pathological and clinical aspects. (cuanschutz.edu)
  • Moreover, diseases related to systemic or local inflammation, including infections, cerebrovascular accidents, and obesity, have been proposed as risk factors for the development of AD. (biomedcentral.com)
  • Other manifestations of immunosuppression, such as the development of autoimmune diseases or malignancies, may also occur with some of these therapies. (medilib.ir)
  • Over the past two decades, the development of targeted immunotherapeutics for relapsing-remitting multiple sclerosis has been successfully orchestrated through the efficacious modulation of neuroinflammatory outcomes demonstrated in the experimental autoimmune encephalomyelitis (EAE) model. (medsci.org)
  • This overall treatment approach should be familiar to neurologists who are accustomed to the evaluation and treatment of other autoimmune conditions such as multiple sclerosis, myasthenia gravis, or polymyositis. (medscape.com)
  • Multiple sclerosis (MS) is a chronic autoimmune disease characterized by inflammation and central nervous system (CNS) demyelination. (arupconsult.com)
  • His research focuses on what provokes relapses and remissions in multiple sclerosis (MS), and on the quest for antigen specific therapy in autoimmune disease. (neuroimmune.org)
  • Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. (e-cep.org)
  • However, current criteria for autoimmune encephalitis. (e-cep.org)
  • The discovery of NMDAR autoantibodies in 2007/8 by Josep Dalmau and his group and the since-emerging recognition and definition of Autoimmune Encephalitis (AE) for a widening spectrum of neurological disorders ( 4 , 5 ) have especially moved the field, because AE can be well diagnosed as a result of presenting with severe neurologic symptoms. (edupony.com)
  • Autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A) is a newly discovered autoimmune nervous system disease involving the brain, spinal cord, meninges and optic nerve. (biomedcentral.com)
  • Autoimmune glial fibrillary acidic protein astrocytopathy is a form of monophasic or relapsing steroid-responsive meningoencephalomyelitis often accompanied by a hallmark radial periventricular brain MRI enhancement pattern and inflammatory cerebrospinal fluid with lymphocytic pleocytosis. (medlink.com)
  • Sjögren's syndrome (SjS) is a common autoimmune disorder associated with epithelial inflammation and exocrine gland dysfunction [ 5 ]. (biomedcentral.com)
  • Sjogren's Syndrome Treatment/Sjogren's syndrome (SS) is an autoimmune disorder caused by the lymphocytic infiltration of exocrine glands resulting in glandular dysfunction, preferentially of the salivary and lacrimal glands [ rx] . (rxharun.com)
  • An extensive evaluation for systemic and infectious etiologies was uninformative (see appendix e-1 at Neurology.org/nn for details). (neurology.org)
  • We believe the answer is probable, since some of the systemic disease clinical manifestations of COVID-19 cannot be explained solely by the binding of SARS-CoV-2 spike proteins with cell membranes of tissues that exhibit angiotensin-converting enzyme 2 (ACE2). (frontiersin.org)
  • 4 , 5 , 7 The estimated frequency of CNS involvement ranges from 10-60% in different reports, depending on the parameters studied (eg, patient selection, diagnostic criteria, etc). 4 ⇓ - 6 Patients with pSS can present with a wide range of focal or diffuse neurologic or psychiatric manifestations, including motor/sensory deficits, transverse myelitis, and cognitive impairment. (ajnr.org)
  • Sjögren's syndrome (SjS) is a relatively common autoimmune disease characterized by oral and ocular dryness. (biomedcentral.com)
  • Whether peripheral neuropathy is a feature of the systemic or glandular disease or whether it is related to a circulating antineuronal antibody remains also uncertain. (bmj.com)
  • Association of the HLA-DPB1*0501 allele with anti-aquaporin-4 antibody positivity in Japanese patients with idiopathic central nervous system demyelinating disorders. (cdc.gov)
  • and (4) discuss the current treatment for DN NMOSD. (bvsalud.org)
  • Both autoimmune disorders, NMOSD and lupus are each characterized by the immune system wrongfully attacking the body's own tissues. (neuromyelitisnews.com)
  • A 68-year-old male Chinese patient presented with headache and fever of up to 39 °C, accompanied by chills, paroxysmal cough and sputum, and systemic myalgia at the end of September 2021. (biomedcentral.com)
  • The ELAC2 gene functions as a mitochondrial tRNA processing gene, and only 4 different pathogenic mutations have been reported in ELAC2 -associated mitochondrial dysfunction involving oxidative phosphorylation. (e-cep.org)
  • Autoimmune and paraneoplastic neurological disorders: A review of relevant neuroimaging findings. (cuanschutz.edu)
  • Association of Aquaporin-3, Aquaporin-7, NOS3 and CYBA polymorphisms with hypertensive disorders in women. (ircabral.com)
  • Liver fibrosis is a complex disease provoked by a range of chronic injuries to the liver, among which are viral hepatitis, (non-) alcoholic steatohepatitis and autoimmune disorders. (biomedcentral.com)
  • Autoimmune GFAP astrocytopathy manifests as a meningoencephalomyelitis (or limited form thereof) with dramatic steroid-responsiveness. (medlink.com)
  • Autoimmune GFAP astrocytopathy presentation is subacute (over days to a few weeks). (medlink.com)
  • Inflammation is known to exacerbate AD pathogenesis [ 4 ]. (biomedcentral.com)
  • External factors, including systemic inflammation, such as that observed in obesity, are likely to interfere with the brain's immune processes and further promote disease progression. (biomedcentral.com)
  • Most important, the early stages of AE are associated with various and varying, initially pure psychiatric syndromes, with neurological symptoms appearing only later in more severe stages, in addition to psychiatric syndromes ( 4 , 5 ). (edupony.com)
  • Ocular symptoms are correlated to systemic disease activity and can present as an initial manifestation of SLE. (bmj.com)
  • 2 Ocular involvement may correlate with systemic disease activity and precede other systemic symptoms stressing the important role the ophthalmologist may play. (bmj.com)
  • We aimed to validate the prevalence of anti-aquaporin 5 (AQP5) IgG in a non-Korean cohort and to optimize the method to screen the anti-AQP5 IgG. (mdpi.com)
  • 4-6 The prevalence of SLE is different between age, gender, geographic and racial distributions. (bmj.com)
  • 4 ⇓ - 6 Although involvement of the peripheral nervous system is a well-documented feature of the disease, the prevalence, the type, and the underlying mechanism of CNS involvement remain unclear. (ajnr.org)
  • The established treatment includes prompt systemic corticosteroids, steroid-sparing immunosuppressive drugs and biological agents. (bmj.com)
  • Sjögren's syndrome is a chronic inflammatory autoimmune disease of unknown origin, attacking, in particular, the tear and salivary glands. (rxharun.com)
  • Numerous clinical trials conducted in participants living with relapsing MS and PPMS have contributed to the understanding of MS pathogenesis allowing for development and management of this heterogeneous disease through targeted disease modifying therapies (DMTs) as the stochastic nature of symptoms evolve [ 4 ]. (medsci.org)
  • However, under the "wrong" environmental circumstances, polyfunctional T-cells(poly-T) can drive autoimmune-disease pathogenesis. (dcu.ie)
  • Accumulation of these mediators culminates in chronic neuroinflammation that exacerbates AD pathogenesis [ 4 , 10 ]. (biomedcentral.com)
  • Idiopathic ON, the most common form of ON, is an inflammatory optic neuropathy in the absence of evidence of a systemic inflammatory disease. (bmj.com)
  • Avian paramyxovirus type 1 (APMV-1) is a virus of birds that results in a range of outcomes, from asymptomatic infections to outbreaks of systemic respiratory and neurologic disease, depending on the virus strain and the avian species affected. (cdc.gov)
  • The pattern of RNFL thinning most specific to MS is temporal.3 RNFL can appear normal in the acute setting but starts to decline within 4 weeks of ON. (clevelandclinic.org)
  • 1, 4, 7, 8 A clinician should further narrow the diagnosis based on the timing of symptoms and associated features (Below). (clevelandclinic.org)
  • In 1845, skin lesions were reported by Hebra and later biopsied in 1872 by Kaposi who also pointed to systemic symptoms. (bmj.com)
  • which, when activated, triggers a secondary autoimmune response. (msdmanuals.com)
  • Sjögren syndrome is a chronic systemic autoimmune disease that can be classified as primary Sjögren syndrome (pSS) when presenting in isolation or secondary when related to another connective tissue disease. (ajnr.org)
  • A comprehensive examination of the eye is often required in patients presenting with systemic complaints. (neurologyindia.com)
  • In the majority of patients, it is a late event and frequently associated with systemic disease or risk factors for lymphoma development. (bmj.com)
  • The functional status and medical fragility of the patient - Studies have shown that hospitalized patients, those with poor functional status or comorbid conditions, and older adults are more likely to develop immune complications when treated with glucocorticoids, and this is likely to be the case for other immunosuppressant drugs [ 4 ]. (medilib.ir)
  • 4 ⇓ - 6 The current data from MRI studies support an increased frequency of high signal intensity lesions in the periventricular and/or subcortical WM on FLAIR and T2-weighted imaging, observed mainly in patients with pSS and evidence of CNS disease. (ajnr.org)
  • This was strongly reinforced by single case reports of successful immune modulatory treatments of cases of Autoimmune Psychosis (AP), with AP cases not fulfilling the diagnostic criteria of AE ( 8 - 13 ). (edupony.com)