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Pediatric Neurology Jul 2021Autoimmune encephalitis (AE) is an increasingly recognized inflammatory disorder of the central nervous system and is most often characterized by antibodies against... (Review)
Review
Autoimmune encephalitis (AE) is an increasingly recognized inflammatory disorder of the central nervous system and is most often characterized by antibodies against intracellular and neuronal surface antigens. AE is a devastating disease that may result in developmental delay or regression in children. However, the pathogenesis of AE is not clear, and immune system disorders after infection likely play an important role in AE. Many studies have reported that patients with herpes simplex virus encephalitis develop anti-N-methyl-d-aspartate receptor encephalitis after antiviral treatment. It is critical to recognize pediatric AE early and to distinguish it from infectious forms because AE is treatable and responsive to immunotherapies. In this review, we discuss the clinical features of pediatric AE and focus on the relationship between AE and postinfection status. In addition, we review the probable mechanisms underlying infection-triggered AE, which include molecular mimicry, bystander activation, epitope spreading, immune system disorder, and genetic susceptibility.
Topics: Autoimmune Diseases of the Nervous System; Child; Encephalitis; Genetic Predisposition to Disease; Humans; Infectious Encephalitis
PubMed: 33964702
DOI: 10.1016/j.pediatrneurol.2021.04.001 -
Autoimmunity Reviews Nov 2023Autoimmune diseases have specific pathophysiologic mechanisms leading to an increased risk of arterial and venous thrombosis. The risk of venous thromboembolism (VTE)... (Review)
Review
Autoimmune diseases have specific pathophysiologic mechanisms leading to an increased risk of arterial and venous thrombosis. The risk of venous thromboembolism (VTE) varies according to the type and stage of the disease, and to concomitant treatments. In this review, we revise the most common autoimmune disease such as antiphospholipid syndrome, inflammatory myositis, polymyositis and dermatomyositis, rheumatoid arthritis, sarcoidosis, Sjogren syndrome, autoimmune haemolytic anaemia, systemic lupus erythematosus, systemic sclerosis, vasculitis and inflammatory bowel disease. We also provide an overview of pathophysiology responsible for the risk of VTE in each autoimmune disorder, and report current indications to anticoagulant treatment for primary and secondary prevention of VTE.
Topics: Humans; Venous Thromboembolism; Autoimmune Diseases; Arthritis, Rheumatoid; Antiphospholipid Syndrome; Lupus Erythematosus, Systemic
PubMed: 37714419
DOI: 10.1016/j.autrev.2023.103447 -
Cells Jul 2023Rheumatoid arthritis (RA) is a highly prevalent, chronic, and progressive autoimmune disorder primarily affecting joints and muscles. The associated inflammation, pain,... (Review)
Review
Rheumatoid arthritis (RA) is a highly prevalent, chronic, and progressive autoimmune disorder primarily affecting joints and muscles. The associated inflammation, pain, and motor restriction negatively impact patient quality of life (QOL) and can even contribute to premature mortality. Further, conventional treatments such as antiinflammatory drugs are only symptomatic. Substantial progress has been made on elucidating the etiopathology of overt RA, in particular the contributions of innate and adaptive immune system dysfunction to chronic inflammation. Although the precise mechanisms underlying onset and progression remain elusive, the discovery of new drug targets, early diagnosis, and new targeted treatments have greatly improved the prognosis and QOL of patients with RA. However, a sizable proportion of patients develop severe adverse effects, exhibit poor responses, or cannot tolerate long-term use of these drugs, necessitating more effective and safer therapeutic alternatives. Mounting preclinical and clinical evidence suggests that the transplantation of multipotent adult stem cells such as mesenchymal stromal/stem cells is a safe and effective treatment strategy for controlling chronic inflammation and promoting tissue regeneration in patients with intractable diseases, including RA. This review describes the current status of MSC-based therapies for RA as well as the opportunities and challenges to broader clinical application.
Topics: Humans; Quality of Life; Arthritis, Rheumatoid; Mesenchymal Stem Cells; Inflammation; Autoimmune Diseases
PubMed: 37508569
DOI: 10.3390/cells12141905 -
Allergy and Asthma Proceedings Sep 2023The pediatric autoimmune neurologic disorders associated with streptococcal infections (PANDAS) comprise a group of patients who, after infection with group A... (Review)
Review
The pediatric autoimmune neurologic disorders associated with streptococcal infections (PANDAS) comprise a group of patients who, after infection with group A β-hemolytic streptococci (GAS), exhibit a spectrum of neuropsychiatric symptoms that include obsessive thoughts, compulsive behaviors, tics, hyperactivity, inattention, and mild choreiform movements. More recently, a group of patients with a symptom complex similar to PANDAS without evidence of streptococcal etiology was given the acronym pediatric acute-onset neuropsychiatric syndrome (PANS). Despite more than several decades of study and increasing numbers of patients being identified with PANDAS and PANS, there are ongoing controversies, which range from disagreements about specific pathogenetic mechanisms to whether these entities actually exist. The purpose of this report was to examine the current body of evidence that deals with the relationship(s) of immunologic host responses to infection and putative immunologic mechanisms involved in the pathogenesis of these disorders, to evaluate the effectiveness of anti-inflammatory and immunomodulatory therapies with intravenous immunoglobulin (IVIG), and to consider the extent to which allergist/immunologists might be involved in their management. An extensive literature review was conducted in medical literature data bases by applying terms such as PANDAS, PAN, autoimmune encephalitis, neuroinflammation, and autoimmune obsessive-compulsive disorders. PANDAS and its later iterative form, PANS, continue to challenge clinicians, patients, and their families. Although the precise reason why these disorders develop remains unknown, both are considered to have an autoimmune basis related to the production of antibodies directed at antigens of the putative causative infectious disease agents that are cross-reactive with antigenic epitopes on selected brain nuclei, which lead to the neuroinflammatory sequelae responsible for the neuropsychiatric symptoms of these conditions, a phenomenon referred to as molecular mimicry. The PANDAS/PANS disorders are a continuing burden for growing numbers of patients, health-care providers, and the global health-care systems, and are a particular challenge for the allergist/immunologist who is increasingly being called upon for their management. Because of the importance of immunologic factors in the pathogenesis and treatment of these conditions with anti-inflammatory and immune-modulating treatments, the allergist/immunologist is well poised to offer consultative care.
Topics: Humans; Child; Allergists; Autoimmune Diseases; Hashimoto Disease; Obsessive-Compulsive Disorder
PubMed: 37641225
DOI: 10.2500/aap.2023.44.230029 -
Seminars in Arthritis and Rheumatism Oct 2022Sjögren's syndrome (SS) is a systemic autoimmune disorder with an estimated global prevalence of 0.3 to 1/1000 persons. This disease has a female predilection and... (Review)
Review
Sjögren's syndrome (SS) is a systemic autoimmune disorder with an estimated global prevalence of 0.3 to 1/1000 persons. This disease has a female predilection and mainly affects salivary and lacrimal glands. The distinctive pathological hallmark of SS is focal lymphocyte infiltration in affected glands, accompanied by the production of autoantibodies and inflammatory cytokines leading to epithelial damage and disease progression. Danger-associated molecular patterns (DAMPs) as alarmins have been demonstrated to promote lymphocyte recruitment in several inflammatory and autoimmune diseases. Here we summarize that the levels of DAMPs were increased in the periphery and affected tissues in SS as the stimulators, DAMPs sensed by pattern recognition receptors (PRRs, the same sensors for PAMPs) initiated the inflammatory and autoimmune response constituting a vicious autoimmunity loop leading to disease exacerbation. Thus, DAMPs are involved in the immunopathogenesis of SS and inhibition of these DAMPs may serve as a novel therapeutic strategy for SS.
Topics: Alarmins; Autoantibodies; Autoimmune Diseases; Female; Humans; Lacrimal Apparatus; Sjogren's Syndrome
PubMed: 35803061
DOI: 10.1016/j.semarthrit.2022.152062 -
Neurological Sciences : Official... Apr 2020Melatonin is a neurohormone mainly produced by the pineal gland following a circadian rhythm. It is characterized as a pleiotropic factor because it not only regulates... (Review)
Review
Melatonin is a neurohormone mainly produced by the pineal gland following a circadian rhythm. It is characterized as a pleiotropic factor because it not only regulates the wake-sleep rhythm but also exerts antinociceptive, antidepressant, anxiolytic, and immunomodulating properties. Recent studies suggest that dysregulation of melatonin secretion is associated with the pathogenesis of various autoimmune diseases, such as, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and multiple sclerosis (MS). MS is an autoimmune disorder characterized by an abnormal immune response directed against the myelin sheath in the central nervous system, demyelination, oligodendrocyte death, and axonal degeneration. Recent evidence reveals that melatonin secretion is dysregulated in MS patients, suggesting that melatonin could be a potential target for therapeutic intervention. Here, we summarize the available literature regarding the role of melatonin in immune processes relevant for experimental autoimmune encephalomyelitis (EAE), MS, and the current clinical trials of melatonin supplementation in MS patients.
Topics: Animals; Encephalomyelitis, Autoimmune, Experimental; Humans; Melatonin; Multiple Sclerosis; Neurotransmitter Agents
PubMed: 31845043
DOI: 10.1007/s10072-019-04137-2 -
Clinical Autonomic Research : Official... Feb 2020
Topics: Autoantibodies; Autoimmune Diseases; Biomedical Research; Heart Rate; Humans; Postural Orthostatic Tachycardia Syndrome
PubMed: 31938977
DOI: 10.1007/s10286-019-00661-5 -
Genes and Immunity Apr 2023Multiple sclerosis is a chronic neuroinflammatory demyelinating disease of the central nervous system (CNS) of unknown etiology and still incompletely clarified... (Review)
Review
Multiple sclerosis is a chronic neuroinflammatory demyelinating disease of the central nervous system (CNS) of unknown etiology and still incompletely clarified pathogenesis. The disease is generally considered a disorder resulting from a complex interplay between environmental risk factors and predisposing causal genetic variants. To examine the etiopathogenesis of the disease, two complementary pre-clinical models are currently discussed: the "outside-in" model proposing a peripherally elicited inflammatory/autoimmune attack against degraded myelin as the cause of the disease, and the "inside-out" paradigm implying a primary cytodegenerative process of cells in the CNS that triggers secondary reactive inflammatory/autoimmune responses against myelin debris. In this review, the integrating pathogenetic role of damage-associated molecular patterns (DAMPs) in these two scenario models is examined by focusing on the origin and sources of these molecules, which are known to promote neuroinflammation and, via activation of pattern recognition receptor-bearing antigen-presenting cells, drive and shape autoimmune responses. In particular, environmental factors are discussed that are conceptually defined as agents which produce endogenous DAMPs via induction of regulated cell death (RCD) or act themselves as exogenous DAMPs. Indeed, in the field of autoimmune diseases, including multiple sclerosis, recent research has focused on environmental triggers that cause secondary events in terms of subroutines of RCD, which have been identified as prolific sources of DAMPs. Finally, a model of a DAMP-driven positive feed-forward loop of chronic inflammatory demyelinating processes is proposed, aimed at reconciling the competing "inside-out" and "outside-in" paradigms.
Topics: Humans; Alarmins; Autoimmune Diseases; Cell Death; Inflammation; Multiple Sclerosis
PubMed: 36750753
DOI: 10.1038/s41435-023-00198-8 -
Alimentary Pharmacology & Therapeutics Jun 2022Inflammatory bowel disease (IBD) is a chronic inflammatory immune-mediated disorder of the gut with frequent extra-intestinal complications. Pancreatic involvement in... (Review)
Review
BACKGROUND
Inflammatory bowel disease (IBD) is a chronic inflammatory immune-mediated disorder of the gut with frequent extra-intestinal complications. Pancreatic involvement in IBD is not uncommon and comprises a heterogeneous group of conditions, including acute pancreatitis (AP), chronic pancreatitis (CP), autoimmune pancreatitis (AIP) and pancreatic exocrine insufficiency (PEI); however, data on such an association remain sparse and heterogeneous.
METHOD
PubMed/MEDLINE and EMBASE databases were searched for studies investigating pancreatic involvement in patients with IBD.
RESULTS
Four thousand one hundred and twenty-one records were identified and 547 screened; finally, 124 studies were included in the review. AP is the most frequent pancreatic manifestation in IBD; the majority of AP cases in IBD are due to gallstones and drugs but cases of idiopathic AP are increasingly reported. AIP is a rare disease, but a strong association with IBD has been demonstrated, especially for type 2 and ulcerative colitis. The pathogenetic link between IBD and AIP remains unclear, but an immune-mediated pathway seems plausible. An association between CP and PEI with IBD has also been suggested, but data are to date scarce and conflicting.
CONCLUSION
This is the first systematic review of the association between IBD and pancreatic diseases. Gallstones and drugs should be considered the most probable causes of AP in IBD, with type 2 AIP also being possible.
Topics: Acute Disease; Autoimmune Diseases; Chronic Disease; Colitis, Ulcerative; Exocrine Pancreatic Insufficiency; Gallstones; Humans; Inflammatory Bowel Diseases; Pancreatitis, Chronic
PubMed: 35505465
DOI: 10.1111/apt.16949 -
Autoimmunity Reviews Jan 2021Autoimmune diseases patients are characterized by the autoimmune disorders, whose immune system can't distinguish between auto- and foreign- antigens. Thus, Immune... (Review)
Review
Autoimmune diseases patients are characterized by the autoimmune disorders, whose immune system can't distinguish between auto- and foreign- antigens. Thus, Immune homeostasis disorder is the key factor for autoimmune diseases development. Adenosine deaminase (ADA) is the degrading enzyme for an immunosuppressive signal - adenosine, and play an important role in immune homeostasis regulation. Increasing evidences have shown that ADA is involved in various autoimmune diseases. ADA activity were changed in multiple autoimmune diseases patients and could be served as a biomarker for clinical diagnosis. In this study, we analyze the change of ADA activity in patients with autoimmune diseases, and we underline its potential diagnostic value for autoimmune diseases patients.
Topics: Adenosine Deaminase; Autoimmune Diseases; Biomarkers; Humans
PubMed: 33197575
DOI: 10.1016/j.autrev.2020.102709