Advances in the Treatment of Behcet’s Disease

Alıbaz-Öner, Fatma; Direskeneli, Haner

doi:10.1007/s11926-021-01011-z

Cited by 25 publications

(14 citation statements)

References 111 publications

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“…Behçet disease is an immune-mediated vasculitis affecting both arteries and veins of any size (small, medium or large) and thus is also termed a variable vessel vasculitis [ 2 , 9 ]. The pathogenesis remains unclear, although an autoinflammatory response involving activation of neutrophils and monocytes and subsequently IL-1 is implicated [ 62 ]. Although Behçet usually manifests after 20 years of age, children can be affected [ 2 ].…”

Section: Other Vasculitismentioning

confidence: 99%

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Pediatric cardiothoracic vasculitis: multimodality imaging review

Chan

2022

Pediatr Radiol

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The pediatric vasculitides are a relatively uncommon and heterogeneous group of disorders characterized by vessel inflammation, often with cardiothoracic involvement. Diagnosis and monitoring are often clinically challenging because of the nonspecific symptoms and laboratory markers. Thus, imaging has assumed increasing importance for early detection of disease activity, extent and complications as well as long-term monitoring pre- and post-treatment. Herein, we review the major pediatric vasculitides with frequent chest manifestations, including Takayasu arteritis, Kawasaki disease, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, Behçet disease and potential mimics. We highlight key clinical features and management considerations, emphasizing the central role of imaging.

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Section: Other Vasculitismentioning

confidence: 99%

“…Characteristic clinical features include recurrent oral or genital aphthous ulcers, skin lesions, uveitis and cardiovascular complications [ 2 , 9 ]. Treatments include glucocorticoids as well as immunosuppressive and biologic agents targeting proinflammatory cytokines [ 62 ].…”

Section: Other Vasculitismentioning

confidence: 99%

Pediatric cardiothoracic vasculitis: multimodality imaging review

Chan

2022

Pediatr Radiol

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“…In one clinical trial of uveitic cystoid macular edema secondary to multiple sclerosis, the IFN-β group had significant improvements in visual acuity and uveitic cystoid macular edema at 3 months [208]. The adverse events of IFN include transaminase elevations, alopecia, thrombocytopenia, leukopenia, depression, and Flulike symptoms [142,210]…”

Section: Interferon (Ifn)mentioning

confidence: 99%

Pathogenesis and current therapies for non-infectious uveitis

Tao

Zhu

et al. 2022

Clin Exp Med

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Non-infectious uveitis (NIU) is a disorder with various etiologies and is characterized by eye inflammation, mainly affecting people of working age. An accurate diagnosis of NIU is crucial for appropriate therapy. The aim of therapy is to improve vision, relieve ocular inflammation, prevent relapse, and avoid treatment side effects. At present, corticosteroids are the mainstay of topical or systemic therapy. However, repeated injections are required for the treatment of chronic NIU. Recently, new drug delivery systems that may ensure intraocular delivery of therapeutic drug levels have been highlighted. Furthermore, with the development of immunosuppressants and biologics, specific therapies can be selected based on the needs of each patient. Immunosuppressants used in the treatment of NIU include calcineurin inhibitors and antimetabolites. However, systemic immunosuppressive therapy itself is associated with adverse effects due to the inhibition of immune function. In patients with refractory NIU or those who cannot tolerate corticosteroids and immunosuppressors, biologics have emerged as alternative treatments. Thus, to improve the prognosis of patients with NIU, NIU should be managed with different drugs according to the response to treatment and possible side effects.

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“…Behçet’s disease (BD) is a chronic relapsing inflammatory disease with a poorly understood etiology, and it is characterized by vasculitis with multisystem involvement including oral-genital-aphthous ulcers, ocular and neurological lesions. 1 – 3 A diagnosis of intestinal BD is established when intestinal symptoms and typical gastrointestinal tract ulcers arise in the defined BD patients. 4 Besides, other typical oval-shaped large ulcers in the terminal ileum were contributory to the diagnosis after the exclusion of other diseases with similar manifestations.…”

Section: Introductionmentioning

confidence: 99%

“…Thus, therapeutic strategies for inflammatory bowel disease are generally prescribed for intestinal BD, including 5-aminosalicylic acids, steroids, and immunomodulators. 1 Nevertheless, not all patients obtain an alleviation from these conventional therapies, and some patients even deteriorate progressively or require surgery. It is reported that the cumulative 5- and 10-year surgery rates for intestinal BD have reached approximately 30 and 40%, respectively.…”

Section: Introductionmentioning

confidence: 99%

Anti-TNF-α agents for refractory intestinal Behçet’s disease: case series and meta-analysis

Zhan

Liu

et al. 2022

Therap Adv Gastroenterol

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Background: Behçet’s disease (BD) is a relapsing systemic immune disorder, and intestinal BD is a significant cause of mortality in patients with BD. Conventional therapeutic strategies for intestinal BD showed unsatisfactory outcomes, especially in those patients with refractory subtypes. In recent years, biologic agents have exhibited promising results in this field. While the sample sizes of existing studies were limited, the results were heterogeneous. Objectives: This study aimed to observe the efficacy of different biologics in clinical symptomatic improvement and intestinal mucosal healing. Design: This is a study including the report of case series and meta-analysis. Data sources and methods: This meta-analysis was conducted following the PRISMA guidelines. Free-text words and subject terms, including ‘Behcet’s Syndrome’, ‘Biologics’, ‘Tumor Necrosis Factor Antagonist’, were used to systematically research the relevant studies in the electronic databases (PubMed, Web of Science, Embase, and Cochrane Library). All retrieved articles were from inception to July 2021, and the data from our institution were also included in this meta-analysis. A double arcsine transformation was performed to stabilize the variance of the original ratio. Heterogeneity was evaluated via Q-test and I2 statistics. Random-effects or fixed-effects model was used to calculate the pooled parameters, and the results were presented as forest plots with 95% confidence intervals. Results: Twelve studies were included, but only antitumor necrosis factor alpha (anti-TNF-α) agents were prescribed as biologicals for refractory intestinal BD. The symptom improvement rates at our institution ranged from 57.1 to 81.8%, and the mucosal healing rates were from 20 to 60% in different therapeutic periods. A total of 514 patients were enrolled in the meta-analysis, and the synthesized ratios showed that 59.8% ( n = 377) and 73.7% ( n = 317) of patients who received maintenance therapy with anti-TNF-α agents could achieve clinical symptomatic improvement during short-term (10–14 weeks) and long-term (48–54 weeks) periods, respectively. Furthermore, 77.8% ( n = 229) of patients with intestinal BD maintained therapeutic efficacy for a longer time (100 weeks). In addition, 60.9% ( n = 120) of the patients achieved sustained intestinal mucosal healing during a long-term follow-up (24–52 weeks). Conclusion: Anti-TNF-α treatment is effective in treating refractory intestinal BD but more studies are required to evaluate the effects of new biologics for intestinal BD in the near future. Registration: This study has been registered on PROSPERO, the ID is CRD42022329211. Plain language summary Anti-TNF-α agents are effective and safe in patients with intestinal Behçet’s disease Behçet’s disease (BD) is a disease affecting several organs including the gastrointestinal tract. Nowadays, the efficacy of existing therapy strategies is still unsatisfactory and some patients are suffering from repeated attacks of the disease. We noticed that a new kind of medicine, called antitumor necrosis factor alpha (anti-TNF-α) agents, was applied to these patients recently. The therapeutic efficacy is not convincing enough to evaluate since the number of patients receiving this new medicine was small in every individual study. Regarding this, we conducted a research to learn about the efficacy of this medicine at our own institution. Besides, we composed the results of other studies in an appropriate way. Then, we drew a conclusion on the exact efficacy of anti-TNF-α agents after the data analysis. We unveiled that the anti-TNF-α agents appeared both effective and safe in the management of intestinal BD patients when the classical therapy failed. More than half of the patients could achieve discomfort remission when they got the therapy of the new medicine at our institution. We also found that intestinal ulcers in most patients improved after they received the treatment. All in all, it offered another foothold for getting relief in these patients who were caught in this mire.

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Advances in the Treatment of Behcet’s Disease

Cited by 25 publications

References 111 publications

Pediatric cardiothoracic vasculitis: multimodality imaging review

Pediatric cardiothoracic vasculitis: multimodality imaging review

Pathogenesis and current therapies for non-infectious uveitis

Anti-TNF-α agents for refractory intestinal Behçet’s disease: case series and meta-analysis

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