Behcets Disease (Inflammatory Disease and Therapy)
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In autoimmune diseases, the frequency of NK cells is decreased and their cytotoxicity is impaired, suggesting that NK cells play a protective role in controlling autoimmunity Hasan et al. Peripheral blood depletion of NK cells in BD patients may reflect increased homing of these cytotoxic cells to inflammatory sites and activation and maintenance of tissue inflammation in BD patients through Th1 cytokine production, resulting in cytotoxicity in the active phase. Cosan et al.
Sutton et al. Parlakgul et al. Neutrophils play a vital role in the innate immune response and are the first line of defense against infectious diseases.
Neutrophils can damage host cells and tissues while destroying microbes. Therefore, tissue damage is one of the main triggers of inflammation, which in turn triggers the immune response Cell surface antigens such as CD10, CD14, and CD16 are expressed in neutrophils and are related to neutrophil function. Hyperactive neutrophils can increase chemotaxis, phagocytosis, and superoxide production 2. The production of reactive oxygen species ROS is a normal characteristic of neutrophils.
Neutrophil-mediated oxidative stress abnormalities may play an important role in the pathogenesis of BD, and advanced oxidation protein products AOPPs , may be a useful marker for monitoring the progression and severity of disease activity in patients with BD BD, also known as chronic vasculitis, is characterized by venous thrombosis, aneurysms and occlusions. Unlike classic vasculitis, pathological studies in BD patients have shown a lack of true necrotizing vasculitis, granuloma, or immunocomplex deposition Histopathological analysis has shown that arteries and veins are infiltrated by neutrophils and lymphocytes, which results in vascular endothelial dysfunction Endothelial dysfunction and neutrophil vascular inflammation are key factors mediating thrombosis in patients with BD 3.
Becatti et al. In particular, their results suggest that an altered fibrinogen structure and impaired fibrinogen function are related to neutrophil activation and the production of enhanced ROS, which are mainly derived from neutrophil NADPH oxidases The Th1 immune response plays an important role in the pathogenesis of BD.
The expression levels of Th1 cells and related cytokines are associated with the activity of BD. Studies have found that the frequencies of Th1 cells and their cytokines and transcription factor T-bet were significantly higher in patients with active BD than those in patients with inactive BD. IL, which is composed of 2 heterodimeric subunits p35 and p40 , is produced by DCs, macrophages and B cells and is a key Th1-inducing cytokine Ahn et al.
In addition, El-Asrar et al. IL is a proinflammatory cytokine that plays an important role in the immune response of Th1 cells. Oztas et al. Furthermore, Musabak et al.
This study suggested that IL is involved in the pathogenesis of BD and that its level is closely related to disease activity Although BD was once considered a Th1-mediated disease, Th17 cells are central in the process of autoimmune diseases. Accumulating evidence suggests that Th17 cells regulate inflammation and autoimmune diseases.
The expression levels of Th17 cells and related cytokines are associated with the activity of BD. Chi et al.
The frequency of circulating Th17 cells in patients with active BD has also been reported to be significantly higher than that in the same patients in the remission stage Jiang et al. IL synergizes with IL-6 to promote the differentiation, survival and maintenance of Th17 cells, and IL can amplify the Th17 cell response by inducing the production of proinflammatory cytokines Therefore, IL plays an important role in the expansion and survival of Th17 cells.
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IL is a member of the IL-2 family of cytokines and its function is mediated by the IL receptor Furthermore, IL is critical for B cell differentiation into plasma cells and antibody class switching via the induction of Blimp-1 and Bcl-6 and negatively regulates the function of DCs 62 , Wang et al. This study suggested that IL is correlated with autoimmune diseases, including BD. Geri et al. This study also suggested the presence of IL and ILA-producing T cells within the cerebrospinal fluid CSF , brain parenchyma inflammatory infiltrates, and intracerebral blood vessels from active BD patients and the involvement of the CNS 67 , Th17 and Treg cells are implicated in inflammatory and autoimmune diseases.
Th17 cells are involved in pathological induction and proliferation, while Treg cells inhibit autoimmunity and develop tolerance to self-antigens Therefore, BD is dominated by the immune response of Th1 and Th17 cells. IL is an inflammatory cytokine that promotes inflammation and is associated with autoimmune diseases. In addition, accumulating evidence suggests that IL plays an important role in the pathogenesis of autoimmune diseases Sugita et al.
In addition, fresh T cells from patients with BD expressed high levels of Threlated molecules. However, when fresh T cells from patients with BD were treated with infliximab, the expression of these molecules was very low. However, other studies have suggested that the IL level in the supernatant of stimulated PBMCs in BD patients with active uveitis was higher than that in patients without uveitis or in normal controls and that the level of IL was associated with the severity of retinal vasculitis and anterior chamber inflammation The increased levels of IL in patients with BD with mucocutaneous lesions might be related to the recurrence of ulcers in the skin and mucosa.
The dynamic proinflammatory and anti-inflammatory changes in IL might be associated with the organ involvement and severity of BD Treg cells specifically express the transcription factor Foxp3 forkhead box protein P3 and highly express surface and intracellular markers, including CD25, GITR, and CTLA-4, which can suppress the activation, proliferation and effector functions of a wide range of immune cells However, data on Treg cell populations in BD patients are contradictory.
In some studies, the ratio of Treg cells was found to be increased in the peripheral blood and CSF 79 , while in other studies, the ratio was found to be decreased Several potential mechanisms for Treg cell suppressive functions have been identified, which are mainly mediated by cell-cell contact, cytokine secretion and metabolic disruption. IL was originally described as a cytokine secreted by Th2 cells. But further studies have shown that IL can also be produced by innate immune cells IL has especially important anti-inflammatory and immunosuppressive effects, such as inhibiting the function of APCs, inducing the differentiation of Treg cells, and controlling the proliferation of other T cell populations Touzot et al.
Liu et al. IL is the most recently identified member of the IL cytokine family and may be a new target for the treatment of autoimmune and inflammatory diseases. IL has an immunosuppressive effect that is achieved via regulatory T and B cells 88 , Recent findings suggest the abnormal expression of IL in inflammatory autoimmune diseases, including EAU. In addition, functional analysis indicates that IL plays a key role in the occurrence and development of autoimmune diseases Sonmez et al. The level of IL in patients with inactive BD and healthy controls was higher than that in patients with active BD, a finding that could be explained by the plasticity between Th17 cells and Treg cells These cytokines have been found in the ocular fluid of patients with BD for more than 20 years and are believed to be the major inflammatory mediators leading to the development of the disease Recent studies have shown that single nucleotide polymorphisms SNPs of these cytokines are associated with the onset of BD and that gene polymorphisms are involved in the pathogenesis of BD, which leads to the increased expression of these proinflammatory cytokines 92 — IL-6 is clearly a pleiotropic cytokine, which is produced by innate immune cells IL-6 production is tightly negatively regulated, and abnormal excessive production of IL-6 has been found to be related to autoimmune and chronic inflammatory diseases The increase in IL-6 in the CSF of patients with neuro-BD has been reported to be associated with long-term prognosis and disease activity and is regarded as a marker of disease activity 98 , IL was first described as an anti-inflammatory cytokine in autoimmune and inflammatory diseases.
Therefore, the level of IL may change in autoimmune and inflammatory diseases Recent studies have shown abnormal IL expression in autoimmune diseases, including Behcet's disease, and functional analysis has shown that IL expression is negatively correlated with the development and pathogenesis of BD , Bouali et al. IL has been shown to have immunosuppressive properties, which can inhibit experimental autoimmune encephalomyelitis EAE and EAU by inhibiting the development or proliferation of Th17 cells and inducing the production of IL — In the absence of ILmediated immunosuppression, the secretion of various inflammatory cytokines is accompanied by severe inflammatory reactions The expression of IL in patients with active BD was reported to be lower than that in normal controls.
In addition, recombinant IL inhibited the differentiation of Th17 cells in both BD patients and healthy controls through the interferon regulatory factor 8 IRF8 pathway The decrease in IL expression was associated with intraocular inflammation in BD, suggesting that IL was involved in the occurrence and development of BD. Despite the traditional options of glucocorticoid and immunosuppressive therapies, the visual outcomes and prognoses of patients with BD have not improved substantially until recently with the advent of biotherapies.
Anti-TNF therapy has been reported to be rapidly effective in inducing and maintaining remission , A retrospective multicenter study in South Korea showed that routine drug treatment was ineffective in 28 patients with intestinal BD, and the clinical effective rate was In a recent phase 2 study, an oral phosphodiesterase 4 inhibitor, apremilast, was found to be very effective in inhibiting oral ulcers For the management of BD, recent studies have shown that apremilast, anakinra, and ustekinumab are effective for the treatment of refractory mucocutaneous involvement.
IL-1 inhibitors and tocilizumab appear to be alternatives for patients with refractory ocular involvement Although the etiology of BD is still unclear, advances in genetics and immunology have led to a better understanding of the immunopathogenesis of BD. Infection-related trigger factors are believed to be involved in the development of BD in patients with a genetic predisposition.
Subsequently, the innate and adaptive immune systems are activated by these trigger factors, resulting in the production of numerous cytokines and chemokines to counteract the antigens and autoantigens. NK cells not only play a cytotoxic role in infected cells and tumor cells but also regulate the function of other immune cells, including DCs and T cells, through the secretion of cytokines. BD is characterized by venous thrombosis, aneurysms and occlusions.
Histopathological analysis has shown that arteries and veins are infiltrated by neutrophils and lymphocytes, which results in vascular endothelial dysfunction. Endothelial dysfunction and neutrophil vascular inflammation are key factors mediating thrombosis in patients with BD. A better understanding of the mechanisms associated with inflammatory responses and adaptive immune system regulation in BD promotes the development of biotherapies.
The clinical application of these biologic agents and their good therapeutic effects in BD are based on our in-depth understanding of the immunopathogenesis of BD. Although biologics to treat BD are expensive and most are still in clinical trials, the treatment effect in patients with refractory BD is worth anticipating. BT carried out the primary literature search, wrote, and revised the manuscript. GS initiated the concept and supervised the writing and revision of the manuscript.
XL and JX were involved in the preparation and revision of the manuscript. All authors read and approved the final manuscript. The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. National Center for Biotechnology Information , U.
Journal List Front Immunol v. Front Immunol. Published online Mar Author information Article notes Copyright and License information Disclaimer. This article was submitted to Autoimmune and Autoinflammatory Disorders, a section of the journal Frontiers in Immunology.
Received Nov 6; Accepted Mar The use, distribution or reproduction in other forums is permitted, provided the original author s and the copyright owner s are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. Abstract Behcet's disease BD is a chronic systemic inflammatory vasculitis of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations.
Introduction Behcet's disease BD is a chronic recurrent multisystemic disease that involves oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, gastrointestinal and central nervous system CNS abnormalities, and other pathologies 1. The Role of Antigens in the Pathogenesis of Behcet's Disease The environmental trigger hypothesis of BD development in patients with genetic susceptibility was proposed many years ago.
Open in a separate window. Figure 1. The role of innate immune cells in the pathogenesis of Behcet's disease. Neutrophil Cells Neutrophils play a vital role in the innate immune response and are the first line of defense against infectious diseases. Figure 2. The role of autoimmune T cells and cytokines in the pathogenesis of Behcet's disease.
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Anti-inflammatory Cytokines in Behcet's Disease IL was first described as an anti-inflammatory cytokine in autoimmune and inflammatory diseases. Figure 3. The latest treatments of Behcet's disease is based on the immunopathogenesis of BD.
Conclusion Although the etiology of BD is still unclear, advances in genetics and immunology have led to a better understanding of the immunopathogenesis of BD. Author Contributions BT carried out the primary literature search, wrote, and revised the manuscript. Conflict of Interest Statement The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
References 1. J Autoimmun. Behcet's disease: ocular effects and treatment. Prog Retinal Eye Res. New insights into the pathogenesis of Behcet's disease. Autoimmun Rev. Gul A, Ohno S. Ocular Immunol Inflamm. Arthritis Rheum. Nat Genet. Expert Opin Invest Drugs. Hedayatfar A. J Ophthalmic Vis Res. Cytokines and Behcet's disease. Immunopathogenesis of ocular Behcet's disease.
J Immunol Res. The role of infectious agents in the pathogenesis, clinical manifestations and treatment strategies in Behcet's disease. Clin Exp Rheumatol. Ann Rheum Dis. Evidence of chronic Chlamydia pneumoniae infection in patients with Behcet's disease. Scand J Infect Dis. J Am Acad Dermatol. Direskeneli H, Saruhan-Direskeneli G. The role of heat shock proteins in Behcet's disease. Clin Exp Immunol. Adamus G, Chan C-C. Experimental autoimmune uveitides: multiple antigens, diverse diseases. Int Rev Immunol. Invest Ophthalmol Vis Sci. S-antigen specific T helper type 1 response is present in Behcet's disease.
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Cell Death Diff. Regulatory NK cells in autoimmune disease. Human natural killer cells: a unique innate immunoregulatory role for the CD56bright subset. Natural killer cells and autoimmunity. Side effects might include headache, skin rash and an increased risk of infections. The unpredictability of Behcet's disease can make it particularly frustrating. Taking good care of yourself might help you cope. In general, try to:. You're likely to start by seeing your primary care doctor. He or she may refer you to a doctor who treats arthritis and other rheumatic illnesses rheumatologist.
Depending on your signs and symptoms, you might also need to see an ophthalmologist for eye problems, a gynecologist or urologist for genital sores, a dermatologist for skin problems, a gastroenterologist for digestive difficulties, or a neurologist for symptoms that involve the brain or central nervous system. Mayo Clinic does not endorse companies or products. Advertising revenue supports our not-for-profit mission. This content does not have an English version. This content does not have an Arabic version. Diagnosis No tests can determine whether you have Behcet's disease, so your doctor will rely primarily on your signs and symptoms.
Request an Appointment at Mayo Clinic. Share on: Facebook Twitter. References AskMayoExpert. Behcet syndrome. Rochester, Minn. Smith EL, et al. Clinical manifestations and diagnosis of Behcet syndrome. Accessed Feb. Behcet's disease. American Behcet's Disease Association. Treatment of Behcet syndrome.