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Understanding Behcet's Disease in the Context of Innate Immunity Activation.

ABSTRACT: Behçet´s disease (BD) is a heterogeneous condition consisting of idiopathic systemic vasculitis affecting large and small blood vessels of different types (i.e., arteries, veins, or capillaries). The disease frequently occurs in young adults without gender predilection, differently from several other autoimmune conditions. This challenging illness has recently been proposed by some authors as an example of complex autoinflammatory syndrome. Although much remains unanswered about BD pathogenesis, recent understanding of some aspects of innate immunity have clarified a few issues (and raised others). HLA-B*51 represents the strongest genetic risk factor for BD to date, albeit several other HLA-independent loci have also been associated with the disease. The consistent hyper-reactivity against Streptococcus sanguinis antigens and alterations in oral and gut microbioma suggests that infectious agents may play an important role. Moreover, functional abnormalities of pattern recognition receptors, especially Toll-like receptors in monocytes, have been demonstrated in patients with BD and can be associated with the development of the disease. Neutrophil hyperactivity is one of the most consistent findings in BD pathogenesis, as demonstrated by exacerbated constitutive oxidative burst, chemotaxis and NET formation. However, some studies suggest that the phagocyte-activated status in BD is not primary to the disease itself, but rather restricted to a fraction of patients with severe disease activity, and probably secondary to activating soluble factors carried by serum/plasma from BD patients. Herein we review the state of the art on BD etiopathogenesis with special emphasis on the participation of the innate immune system.

SUBMITTER: Perazzio SF

PROVIDER: S-EPMC7606308 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Understanding Behçet's Disease in the Context of Innate Immunity Activation.

Perazzio Sandro F SF Andrade Luis E C LEC de Souza Alexandre W S AWS

Frontiers in immunology 20201020

Behçet´s disease (BD) is a heterogeneous condition consisting of idiopathic systemic vasculitis affecting large and small blood vessels of different types (i.e., arteries, veins, or capillaries). The disease frequently occurs in young adults without gender predilection, differently from several other autoimmune conditions. This challenging illness has recently been proposed by some authors as an example of complex autoinflammatory syndrome. Although much remains unanswered about BD pathogenesis, ...[more]

PMID: 33193413

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Project description:BackgroundBehçet's syndrome is a multisystemic vasculitis of unknown aetiology. Cardiac involvement is rare, with described prevalence between 1% and 46%, with pericarditis, valvular insufficiency, intracardiac thrombosis, and eventually sinus of Valsalva aneurysms being the most common findings. Although previously reported, myocarditis is a very rare complication of Behçet's syndrome.Case summaryA 26-year-old man, smoker but otherwise healthy, was admitted to the emergency department with atypical chest pain, with no radiation, relation to efforts, position or deep inspiration, and dyspnoea, since the day before. His physical examination was unremarkable, including no fever, tachycardia, or pericardial friction rub. Electrocardiogram (ECG) revealed an early repolarization pattern, with no changes noted in subsequent exams. He had elevation of inflammatory parameters and an increased high-sensitivity troponin level of 3300 ng/L. Transthoracic echocardiography (TTE) was unremarkable. Coronary angiography showed no coronary stenosis. A presumed diagnosis of non-complicated viral myocarditis was established. The patient's condition improved with acetylsalicylic acid as needed and colchicine and he was discharged after 3 days. Cardiac magnetic resonance was performed, showing late epicardial enhancement in the apical segment of the lateral wall, supporting the diagnosis of myocarditis. Four months later, the patient returned with recurrence of chest pain. Additionally, he also complained of fever, odynophagia, and otalgia since the previous week. Oropharyngeal examination revealed tonsillar pillars aphthosis. The ECG was similar to the previous and TTE was normal. Bloodwork revealed once again elevation of inflammatory parameters and elevation of troponin. Recurrent myocarditis was diagnosed. Treatment with ibuprofen, colchicine, and antibiotic therapy was started with no significant improvement. After a more thorough physical examination, an ulcerated scrotal lesion, a left buttock folliculitis, and an axillary hidradenitis were found, which, according to the patient, were recurrent in the last year. Accordingly, the diagnosis of Behçet's syndrome with mucocutaneous and cardiac involvement was established. The patient was kept on colchicine and was also started on immunosuppressive therapy with corticosteroids and azathioprine, with resolution of the symptoms in the following day. A positron emission tomography (PET) was performed 2 days after discharge and showed a higher myocardial uptake in the left ventricular basal segments and both papillary muscles. Prednisolone tapering was started after 2 months, while maintaining azathioprine. At 1-year follow-up, the patient remained asymptomatic. A re-evaluation PET was performed, showing no images suggestive of metabolically active disease in the myocardium.DiscussionThis case highlights the importance of awareness of this rare but potentially serious entity and reinforces the significance of aetiology investigation in cases of recurrent myocarditis. It also shows the success of immunosuppressive therapy in a context where the optimal management is still considerably uncertain.

Dataset Information

Understanding Behcet's Disease in the Context of Innate Immunity Activation.

Publications

Understanding Behçet's Disease in the Context of Innate Immunity Activation.

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