Refractory Behçet’s Disease with Multi-organ Involvement - learning from Failure

2020 ◽  
Vol 16 ◽  
Author(s):  
Christiana Sieiro Santos ◽  
Clara Moriano Morales ◽  
Elvira Díez Álvarez ◽  
Trinidad Pérez Sandoval
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Organ Involvement ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Aphthous Ulcers ◽  
Early Implementation ◽  
Symptom Complex

Abstract:: Behçet’s disease (BD) is a rare systemic vasculitis of unknown etiology with relapsing and remitting course, characterized by triple-symptom complex of recurrent attacks of oral aphthous ulcers, genital ulcers and ocular lesions and other clinical features that include dermatological, cardiovascular, gastrointestinal, and neurological manifestations. The main goal of management is to prevent relapses and to suppress inflammation rapidly for major organ involvement that may cause damage and even be fatal. We hereby describe a case of patient with Behçet’s disease followed in our rheumatology consultation over the course of 15 years with multi-organ involvement that was resistant to several treatments and showed partial results with IL-1 inhibitors, particularly anakinra and canakinumab, while also discussing the current treatments of refractory BD and how early implementation of treatments could make a difference.

scholarly journals Aortic thrombosis in a patient with a late diagnosis of Behcet's disease

2015 ◽  
Vol 14 (2) ◽  
pp. 193-196
Author(s):  
Ana Bittencourt Detanico ◽  
Marcelo Luiz Brandão ◽  
Ly de Freitas Fernandes ◽  
Carolina Parreira Ribeiro Camelo ◽  
Juliano Ricardo Santana dos Santos
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Thromboangiitis Obliterans ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Oral Ulcers ◽  
Arterial Involvement ◽  
Genital Ulcers

Behcet's disease is form of systemic vasculitis of unknown etiology. One surprising feature is that arterial involvement is less common in this disease than venous forms, accounting for 1 to 7% of patients. In 7 to 30% of cases the vascular symptoms precede the clinical diagnosis of Behcet. We describe the case of a patient with Thromboangiitis Obliterans who was treated with lumbar sympathectomy and an aortobiiliac bypass. Around 1 year later he was diagnosed with Behcet's disease after presenting with oral ulcers, genital ulcers and scaling lesions on the hands.

scholarly journals Isomer-Specific Monitoring of Sialylated N-Glycans Reveals Association of α2,3-Linked Sialic Acid Epitope With Behcet’s Disease

2021 ◽  
Vol 8 ◽  
Author(s):  
Nari Seo ◽  
Hyunjun Lee ◽  
Myung Jin Oh ◽  
Ga Hyeon Kim ◽  
Sang Gil Lee ◽  
...  
Keyword(s):  
Sialic Acid ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Multiple Reaction Monitoring ◽  
High Sensitivity ◽  
High Specificity ◽  
Unknown Etiology ◽  
Behcet's Disease

Behcet’s disease (BD) is an immune disease characterized by chronic and relapsing systemic vasculitis of unknown etiology, which can lead to blindness and even death. Despite continuous efforts to discover biomarkers for accurate and rapid diagnosis and optimal treatment of BD, there is still no signature marker with high sensitivity and high specificity. As the link between glycosylation and the immune system has been revealed, research on the immunological function of glycans is being actively conducted. In particular, sialic acids at the terminus of glycoconjugates are directly implicated in immune responses, cell–cell/pathogen interactions, and tumor progression. Therefore, changes in sialic acid epitope in the human body are spotlighted as a new indicator to monitor the onset and progression of immune diseases. Here, we performed global profiling of N-glycan compositions derived from the sera of 47 healthy donors and 47 BD patients using matrix-assisted laser desorption/ionization-time of flight mass spectrometry (MALDI-TOF MS) to preferentially determine BD target glycans. Then, three sialylated biantennary N-glycans were further subjected to the separation of linkage isomers and quantification using porous graphitized carbon-liquid chromatography (PGC-LC)/multiple reaction monitoring (MRM)-MS. We were able to successfully identify 11 isomers with sialic acid epitopes from the three glycan compositions consisting of Hex5HexNAc4NeuAc1, Hex5HexNAc4Fuc1NeuAc1, and Hex5HexNAc4NeuAc2. Among them, three isomers almost completely distinguished BD from control with high sensitivity and specificity with an area under the curve (AUC) of 0.945, suggesting the potential as novel BD biomarkers. In particular, it was confirmed that α2,3-sialic acid at the terminus of biantennary N-glycan was the epitope associated with BD. In this study, we present a novel approach to elucidating the association between BD and glycosylation by tracing isomeric structures containing sialic acid epitopes. Isomer-specific glycan profiling is suitable for analysis of large clinical cohorts and may facilitate the introduction of diagnostic assays for other immune diseases.

scholarly journals Mucocutaneous Involvement in Behçet’s Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Cinzia Rotondo ◽  
Giuseppe Lopalco ◽  
Florenzo Iannone ◽  
Antonio Vitale ◽  
Rosaria Talarico ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Current State ◽  
Life Threatening ◽  
Symptom Complex ◽  
Appropriate Therapy

Behçet’s disease (BD) is a multisystemic disorder of unknown etiology characterized by the “triple symptom complex” consisting of recurrent oral aphthosis, genital ulcers, and chronic relapsing bilateral uveitis. Recurrent mucocutaneous lesions are generally considered the hallmark of the disease, being the most common symptoms presenting at the onset of disease. Although the improvement of knowledge about the pathogenetic mechanism added important changes in the treatment management of BD clinical manifestations, thus avoiding the appearance of serious life-threatening complications which are disease related, the mucocutaneous lesions are still the most nagging clinical manifestations to be treated. In this work we reviewed the current state of knowledge regarding the therapeutic approaches for mucocutaneous lesions of BD mainly based on controlled studies to provide a rational framework for selecting the appropriate therapy for treating these troublesome features of the disease.

Behcet's Disease

2019 ◽  
Vol 48 (1) ◽  
pp. 103-105
Author(s):  
David S Williams
Keyword(s):  
Behçet’S Disease ◽  
Acute Inflammation ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
The Body ◽  
Unknown Etiology ◽  
Behcet's Disease

Behcet's disease is a rare systemic vasculitis disorder of unknown etiology characterized by recurrent attacks of acute inflammation affecting multiple parts of the body.

Behcet's Disease

2017 ◽  
Vol 107 (5) ◽  
pp. 454-456
Author(s):  
James M. Mahoney ◽  
Kelsey Millonig
Keyword(s):  
Lower Extremity ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Arterial Disease ◽  
Joint Involvement ◽  
Behcet's Disease ◽  
Symptom Complex

Behcet's disease is a rare autoimmune systemic vasculitis. It usually presents with a symptom complex involving primarily mucocutaneous lesions, genital lesions, and uveitis. When it involves the lower extremity, venous and arterial disease predominates, and joint involvement occurs in approximately 50% of patients. We present a patient with Behcet's disease who was initially referred to us for chronic toenail pathology.

scholarly journals The Immediate Pain-Relieving Effects of Non-Thermal CO2 Laser Therapy on Genital Ulcers of Behcet’s Disease: A Case Report

2020 ◽  
Vol 11 (1) ◽  
pp. 108-111
Author(s):  
Nasrin Zand ◽  
Parvin Mansouri ◽  
Samad Rezaee Khiabanloo ◽  
Mohsen Fateh ◽  
Leila Ataie-Fashtami ◽  
...  
Keyword(s):  
Laser Therapy ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Negative Impact ◽  
Systemic Vasculitis ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Genital Ulcers ◽  
Multiple Treatments

Behçet’s disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology. Oral and genital aphthous ulcers are considered as the hallmarks of BD. The genital ulcers of BD may be extremely painful and often refractory to multiple treatments. In addition, they exert a negative impact on the patient’s quality of life. Some investigations have demonstrated the significant and immediate pain-relieving effects of NTCLT (non-thermal CO2 laser therapy) on some oral lesions with no visible adverse effects. In this paper, we report a case of BD whose painful genital ulcers were irradiated with NTCLT and the patient’s pain caused by the ulcers relieved immediately and significantly with no visible complications.

scholarly journals Old and New Challenges in Uveitis Associated with Behçet’s Disease

2021 ◽  
Vol 10 (11) ◽  
pp. 2318
Author(s):  
Julie Gueudry ◽  
Mathilde Leclercq ◽  
David Saadoun ◽  
Bahram Bodaghi
Keyword(s):  
Behçet’S Disease ◽  
Structural Damage ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Retinal Vasculitis ◽  
Unknown Origin ◽  
Current Data ◽  
Ocular Involvement ◽  
Behcet's Disease

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.

scholarly journals Immunopathogenesis of Ocular Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Inflammatory Disorder ◽  
General Hypothesis ◽  
Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

Clinical Findings, Diagnosis, and Classification of Behçet's Disease

2018 ◽  
pp. 329-333
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Clinical Findings ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Behcet's Disease ◽  
Diagnosis And Classification

Behçet’s Disease (BD) is a chronic multisystem vasculitis with unknown etiology, which is characterized by the triad of recurrent oral ulcers, genital ulcers, and iritis. The eye is one of the most commonly affected organs and typical ocular involvement is panuveitis with hypopyon and retinal vasculitis. The diagnosis is based on clinical evaluation including biomicroscopic and funduscopic examination. This article aims to review the clinical findings, diagnosis, and classification of BD.

scholarly journals Pulmonary embolism in Behcet’s disease: a case report

2020 ◽  
Vol 90 (4) ◽  
Author(s):  
Despoina Moumtzi ◽  
Marianna Kakoura
Keyword(s):  
Pulmonary Embolism ◽  
Differential Diagnosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Rare Complication ◽  
Pulmonary Involvement ◽  
Unknown Etiology ◽  
Disease Exacerbation ◽  
Behcet's Disease

Behcet’s disease (BD) is a vasculitis of unknown etiology. It is often correlated with thrombophilic factors such as V Leiden. Pulmonary involvement is reported in 1-10% of patients. The most common manifestations are pulmonary aneurysms while pulmonary embolism is a rare complication. A 41-year old man with BD and V Leiden heterozygosity complained of pleurodynia and fever. Pleurodynia deteriorated in the following days and PE was confirmed by CT angiography, without the presence of aneurysms. After the exclusion of the antiphospholipid syndrome, a therapeutic dose of apixaban was initiated. Two weeks later, pleurodynia relapsed in combination with pleural effusion unilaterally. These findings were attributed to disease exacerbation. For this reason, we decided to enhance the immunosuppressive therapy. Six months later, CTPA showed complete remission of the clots. Vasculitis predisposes to thrombosis with or without coexisting thrombophilia. Clinicians should include them in their differential diagnosis and provide personalized treatment, based on immunosuppressants.

Sign in / Sign up

Export Citation Format

Share Document