scholarly journals Association of Behçet disease with psoriasis and psoriatic arthritis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Hyung Jin Hahn ◽  
Sang Gyu Kwak ◽  
Dong-Kyu Kim ◽  
Jong-Yeup Kim
Keyword(s):  
Psoriatic Arthritis ◽  
Systemic Vasculitis ◽  
Behçet Disease ◽  
Inflammatory Disorder ◽  
Behcet Disease ◽  
Cutaneous Manifestations ◽  
Increased Risk ◽  
Lethal Complications ◽  
Male Cohort ◽  
Clinical Pictures

AbstractBehçet disease (BD) is a debilitating multi-systemic vasculitis with a litany of muco-cutaneous manifestations and potentially lethal complications. Meanwhile, psoriasis (PSO) is a cutaneous and systemic inflammatory disorder marked by hyperplastic epidermis and silvery scales, which may be accompanied by a distinct form of arthropathy called psoriatic arthritis (PsA). While the clinical pictures of these two are quite different, they feature some important similarities, most of which may stem from the autoinflammatory components of BD and PSO. Therefore, the aim of this study was to investigate the prospective link between BD and cutaneous and articular manifestations of psoriasis. BD, PSO, and PsA cohorts were extracted using the National Health Insurance Service of Korea database. Using χ2 tests, prevalence of PSO and PsA with respect to BD status was analysed. Relative to non-BD individuals, those with personal history of BD were nearly three times more likely to be diagnosed with PSO. The adjusted odds ratio (aOR) was 2.36 [95% confidence interval (CI), 1.91–2.93, p < 0.001]. Elevated PSO risk was more pronounced in the male BD cohort (aOR = 1.19, 95% CI 1.16–1.23, p < 0.001). In age-group sub-analysis, individuals over 65 years with PSO were one and a half times more likely to be affected with BD, relative to those under 65. The adjusted OR for the older group was 1.51 (95% CI 1.43–1.59, p < 0.001). BD individuals with “healthy” body weight were significantly less likely to be affected by PSO (aOR = 0.59, 95% CI 0.57–0.62, p < 0.001). On the other hand, there was a correlation between BMI and the risk of BD, with the “moderately obese (30–35 kg/m2)” group having an aOR of 1.24 (95% CI 1.12–1.38, p < 0.001). BD patients were also twice more likely to be associated with PsA (aOR = 2.19, 95% CI 1.42–3.38, p < 0.001). However, in contrast to the case of psoriatic disease itself, females were exposed to a greater risk of developing BD compared to the male PsA cohort (aOR = 2.02, 95% CI 1.88–2.16, p < 0.001). As with PSO, older BD patients were exposed to a significantly higher risk of developing PsA (aOR = 3.13, 95% CI 2.90–3.40, p < 0.001). Behçet disease may place an individual at a significantly increased risk of psoriasis, and still greater hazard of being affected with psoriatic arthritis. This added risk was pronounced in the male cohort, and tended to impact senile population, and this phenomenon may be related with the relatively poor prognosis of BD in males and PSO in older patients.

scholarly journals THU0590 WHEN RARE IS EVEN RARER: A COMPLEX CASE OF BEHCET DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 537.3-537
Author(s):  
M. DI Cicco ◽  
O. M. Epis ◽  
C. Casu ◽  
A. Adinolfi ◽  
L. Alvaro ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Vascular Complications ◽  
Artery Aneurysm ◽  
Skin Lesions ◽  
Complex Case ◽  
Behçet Disease ◽  
Inflammatory Disorder ◽  
X Rays ◽  
Behcet Disease ◽  
Intracardiac Thrombosis

Background:Behcet disease is a rare inflammatory disorder with the unique ability to affect vessels of any size. The disease could be associated to thrombosis in both the venous and arterial compartment, and often aneurysms. In particular, the presence of aneurysms of the pulmonary artery is rarely, if ever, seen in conditions other than Behcet. Cardiac involvement, albeit uncommon, is also described and associated to a severe prognosis. The treatment is based on immunosuppressants, meanwhile the use of anticoagulants -especially when aneurysms are present- is debated.Objectives:To describe a complex case of Behcet disease.Methods:We report the case of a 45 years old man of Chinese origin who presented to A&E with fever and acute dyspnea. Blood test revealed raised ESR and CRP and raised neutrophil count. Chest X rays showed bilateral opacities suggesting pneumonia. The patient did not improve over the course of antibiotics. Later on, he presented with an episode of hemoptysis and worsening dyspnea, so he was admitted to the Intensive Care Unit. CT showed bilateral pulmonary thromboembolism and aneurysm of the pulmonary artery. Echocardiogram and cardio-MRI revealed a large, mobile thrombus within the right atrium. Extensive work-up for infections and cancer was unrevealing. ANA, ENA and ANCA antibodies were negative. On the basis of a past medical history of recurrent oral ulcers and papulopustular skin lesions that patient admitted on questioning, a diagnosis of Behcet disease was suspected. In keeping with that, HLA-B51 turned out positive. The patient was promptly started on IV steroid pulses followed by Cyclophosphamide 1 gr IV monthly for six months, then on IV anti-TNF alpha Infliximab. He was also commenced on low molecular weight heparin (LMWH) and subsequently direct factor Xa inhibitor Apixaban.Results:The patient improved significantly with progressive regression of the pulmonary CT changes. He was discharged and able to get back to his daily life activities. After 2 years and a half of treatment, the aneurysm was stable and the intracardiac thrombus completely cleared.Conclusion:This case is of particular interest because of the concomitant presence of two rare vascular complications of Behcet disease-intracardiac thrombosis (<1-2%, less than 100 cases described worldwide) and pulmonary artery aneurysm (1-2%). Prompt introduction of immunosuppressant therapy was associated with a favorable outcome with no recurrence. We could speculate that, to some extent, the concomitant use of anticoagulants may have contributed to the complete resolution of the intracardiac thrombosis.Disclosure of Interests :MARIA DI CICCO: None declared, oscar massimiliano epis Consultant of: yes, Speakers bureau: yes, Cinzia Casu: None declared, Antonella Adinolfi: None declared, Luisa Alvaro: None declared, Valeria Campanella: None declared, Michel Chevallard: None declared, Marina Muscarà: None declared, Mariaeva Romano: None declared, Emanuela Schito: None declared, Nicola Ughi: None declared, Elisa Verduci: None declared, Davide Antonio Filippini: None declared

Macroscopic features of knee synovitis in early untreated Behçet disease and psoriatic arthritis

2009 ◽  
Vol 28 (9) ◽  
pp. 1053-1057 ◽  
Author(s):  
Concepción Moll ◽  
Mónica Bogas ◽  
José A. Gómez-Puerta ◽  
Raquel Celis ◽  
Ivonne Vázquez ◽  
...  
Keyword(s):  
Psoriatic Arthritis ◽  
Behçet Disease ◽  
Behcet Disease

scholarly journals The Effects of Tumor Necrosis Factor Inhibitors in a Particular Association of Psoriatic Arthritis and Behcet Disease

2019 ◽  
Vol 70 (3) ◽  
pp. 1067-1070
Author(s):  
Anca Emanuela Musetescu ◽  
Alesandra Florescu ◽  
Ana-Maria Bumbea ◽  
Lucian Mihai Florescu ◽  
Paulina Lucia Ciurea ◽  
...  
Keyword(s):  
Psoriatic Arthritis ◽  
Tumor Necrosis ◽  
Behçet Disease ◽  
Complex Treatment ◽  
Behcet Disease ◽  
Current Case ◽  
Disease Modifying Drugs ◽  
Disease Modifying ◽  
Tuberculosis Therapy ◽  
Necrosis Factor

The current case report presents a patient diagnosed with Behcet disease in concurrence with psoriatic arthritis, leading to a complex treatment, difficult management and challenging approach of both rheumatic disorders. After treatment with synthetic disease-modifying drugs and three different TNF inhibitors, the patient developed pulmonary tuberculosis, followed by tuberculosis spondylodiscitis, even after proper anti-tuberculosis therapy.

scholarly journals Cardiac complication spectrum of Behçet disease in Tunisia: A 10-case series: Clinical and therapeutic approach

2020 ◽  
Author(s):  
Ahmed Sami HAMMAMI ◽  
Mohamed JELLAZI ◽  
Marwa BEN BRAHIM ◽  
Syrine DAADA ◽  
Majed HASSINE ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Coronary Artery ◽  
Severe Heart Failure ◽  
Case Series ◽  
Behçet Disease ◽  
Imaging Features ◽  
Behcet Disease ◽  
Adequate Treatment ◽  
Increased Risk ◽  
Artery Disease

Abstract Introduction:Cardiac involvement (CI), although rare, remains one of the most severe complications of Behçet Disease (BD).Objectiveto investigate the frequency and spectrum of cardiac involvement in BD and to assess the clinical and imaging features, treatment, and outcomes.MethodsWe retrospectively retrieved the medical records of patients with CI among 220 BD patients admitted to the internal medicine department between 2006 and 2016 who fulfilled the International Study Group diagnostic criteria for the classification of BD.ResultsTen patients were eligible for the study with a sex ratio (male/female) of 8/10. Three cases had 2 isolated episodes of cardiac BD. The mean age when diagnosing the first cardiac episode was 37,3-year-old. The different types of CI were: coronary artery disease (5/10), intracardiac thrombus (4/10) myocarditis (1/10), pericarditis (1/10), myocardial fibrosis (1/10). Vascular involvement was associated to CI in 5 cases (50%). The main symptoms were chest pain (80%), fever (60%), dyspnea (50%), cough (10%) and hemoptysis (10%). The laboratory tests revealed increased inflammatory markers in 5 patients. Medical treatment was based on colchicine and corticosteroid in all patients (100%), anticoagulants in 8 patients (80%), Cyclophosphamide followed by Azathioprine in 9 patients (90%), Azathioprine monotherapy was started in one patient (10%). Antiplatelet and anti-ischemic therapy in 5 patients who had coronary artery disease. The evolution was favorable in 9 cases and marked by severe heart failure and the death of one patient.ConclusionCI despite its rarity, remains an important feature of BD due to the increased risk of mortality and morbidity. Thus, early screening and detection are paramount. Also, imaging has been of great contribution to diagnose such complications and hence indicate the adequate treatment, including immunosuppressant agents.

scholarly journals Insertion/deletion polymorphism of the ACE gene increased risk of Behcet disease: evidence from a meta-analysis

2013 ◽  
Vol 33 (5) ◽  
pp. 437-442 ◽  
Author(s):  
Raju Kumar Mandal ◽  
Suraj Singh Yaday ◽  
Aditya K. Panda ◽  
Sanjay Khattri
Keyword(s):  
Meta Analysis ◽  
Behçet Disease ◽  
Behcet Disease ◽  
Deletion Polymorphism ◽  
Ace Gene ◽  
Increased Risk

scholarly journals Cytokines Modulate the “Immune-Metabolism” Interactions during Behçet Disease: Effect on Arginine Metabolism

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Houda Belguendouz ◽  
Karima Lahmar-Belguendouz ◽  
Djamel Messaoudene ◽  
Zineb Djeraba ◽  
Fifi Otmani ◽  
...  
Keyword(s):  
Mononuclear Cells ◽  
Clinical Findings ◽  
Subsequent Treatment ◽  
Behçet Disease ◽  
Inflammatory Disorder ◽  
Behcet Disease ◽  
Peripheral Blood Mononuclear ◽  
Chronic Inflammatory Disorder ◽  
Disease Effect ◽  
Different Response

Aim and Methods. In this study, we evaluated NOS and arginase activities and their regulation during Behçet disease, a systemic chronic inflammatory disorder with uncertain etiology. The peripheral blood mononuclear cells of 36 patients and 15 control samples (PBMC) were cultured in either RPMI 1640, MEM, or DMEM complemented with 10% of FBS and antibiotics. Cultures were performed with or without the control or patients plasma. Subsequent treatment contained anticytokines (IL-6, TGF-β), a mitogenic effector (PHA), or NOS modulators (L-NMMA, BH4). Culture supernatants were harvested after 24 h of incubation. NO and urea measurements were, respectively, performed by modified Griess and Berthelot methods.Results. Higher urea levels were found in patients’ plasma compared to the control’s (P< 0.05). NOS modulators induced inverted production profiles for NO and urea (P< 0.05). Their results differed depending on the clinical findings (P< 0.05). It was also found that cytokine neutralization induced different response profiles in patients as opposed to control cultures (P< 0.05).Conclusion. Our results suggest that arginases can compete with NOS2 for L-arginine during Behçet disease. Both enzymes are regulated by environmental cytokines and substrate availability. Furthermore, it seems that NOS/arginase balance is dependent on clinical expression.

Regulatory T cell/Th17 balance in the pathogenesis of pediatric Behçet disease

Rheumatology ◽  
2021 ◽  
Author(s):  
Anne Filleron ◽  
Tu Anh Tran ◽  
Audrey Hubert ◽  
Alexia Letierce ◽  
Guillaume Churlaud ◽  
...  
Keyword(s):  
T Cells ◽  
T Cell ◽  
T Cell Response ◽  
Behçet Disease ◽  
Inflammatory Disorder ◽  
Behcet Disease ◽  
Regulatory T Lymphocytes ◽  
Hla Dr ◽  
Cell Subpopulations ◽  
T Cell Subpopulations

Abstract Objectives Behçet disease (BD) is a chronic systemic inflammatory disorder of unknown aetiology. The aim of this study was to determine the orientation of T cell subpopulations in pediatric BD and more precisely to look for a regulatory T lymphocytes (Tregs)/Th17 imbalance. Methods T cell subpopulations were analyzed by flow cytometry in the peripheral blood of pediatric patients with acute (aBD, n = 24), remitting (rBD, n = 12) BD, and in healthy controls (HC, n = 24). Tregs (CD4+CD25hiCD127-/loFoxp3+), activated Tregs (GITR, LAP, CTLA-4, and HLA-DR expression), CD4+ and CD8+ T cells producing interferon-g (Th1 and Tc1) or interleukin (IL)-17 (Th17 and Tc17) under polyclonal (OKT3/IL-2) or antigenic (Streptococcus sanguis KTH-1 peptides and HSP-60) stimulation, were numerated. Results Th17 (1.9 and 5.1 fold) and Tc17 (4.0 and 2.0 fold) frequency under mitogenic stimulation was significantly increased in aBD and rBD patients as compared with HC. Th17 frequency under antigenic stimulation was also higher in patients than in HC. The percentage and number of Tregs and activated Tregs in patients and in HC were similar. However, when Tregs were removed, antigen-driven differentiation into Th1 and Th17 was significantly boosted in BD but not in HC CD4+T cells. Conclusion There is a bias toward a Th17 polarization in acute and remitting BD children. Although we did not observe an increase in the number of Tregs in these patients, their Tregs limit CD4+T cell differentiation into Th1 and Th17 cells. Thus, in pediatric BD, Tregs seem to incompletely counterbalance a Th17 orientation of the helper T cell response.

scholarly journals Comment on “Behçet disease: a rare systemic vasculitis in Poland”: authors’ reply

2017 ◽  
Vol 127 (11) ◽  
pp. 803-804
Author(s):  
Krzysztof Kanecki ◽  
Aneta Nitsch‑Osuch ◽  
Paweł Goryński ◽  
Patryk Tarka ◽  
Anna Kutera ◽  
...  
Keyword(s):  
Systemic Vasculitis ◽  
Behçet Disease ◽  
Behcet Disease

scholarly journals Penyakit Adamantiades-Behcet Koinfeksi dengan Herpes Genital

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Charly M. M. Korompis ◽  
Triomega F. X. Sengkey ◽  
Shienty Gaspersz ◽  
J. Niode
Keyword(s):  
Genital Herpes ◽  
Herpes Genitalis ◽  
Behçet Disease ◽  
Inflammatory Disorder ◽  
Behcet Disease ◽  
Genital Area ◽  
Pathergy Test ◽  
Irregular Border ◽  
Multiple Ulcers ◽  
Hsv 1

Abstract: Adamantiades-Behcet (AB) disease is a rare multi-systemic inflammatory disorder with unknown cause. It is characterized by recurrent mucocutaneous ulcer in the mouth and genitalia, and mostly affects the age group of 20 to 30 ys. Genital lesion of AB must be differentiated from others caused by sexually transmitted disease, such as genital herpes. Co-infection of AB with genital herpes is very rare. We reported a male 72-year old, came with painful ulcers in the mouth and genital area since a week before visit. Symptoms were recurrent since three years ago. History of arthritis, recurrent headache, eye and skin lesions were denied. Patient was sexually promiscuous. Physical examination revealed multiple ulcers on the labium oris sized 0.5cm-1cm, irregular border, base covered by fibrin tissue, associated with erosion and crust. Multiple ulcers were also found on penile glans, sized 0.1x1x0.2 cm, irregular border, with pus and necrotic tissue. The ulcers were punched out. Pathergy test and anti HSV-1 IgM were negative meanwhile anti HSV-1 IgG, anti HSV-2 IgM as well as anti HSV-2 IgG were positive. Acyclovir 200mg 5x/day for five days, triamcinolone acetonide lotion bid for the mouth ulcer, NaCl 0,9% dressing applied tid for 30 minutes, and fucidic acid cream bid were given and the symptoms improved after 10 days. The prognosis was bonam for ad vitam and dubia for ad functionam and ad sanationam. Conclusion: The diagnosis of AB was based on the International Criteria for Behcet Disease, with a total score of 4 for the recurrent ulcer in mouth and genital area. The positive result of anti HSV-1 IgG, anti HSV-2 IgM and anti HSV-2 IgG supported the coinfection with genital herpes. This coinfection of AB and genital herpes was the first reported in Manado. Immunosenescence was a possible risk factor of the recurrent genital herpes. Symptomatic and antiviral treatment improved the symptoms with possible recurrent genital herpes.Keywords: Adamantiades-Behcet, genital herpes, coinfectionAbstrak: Penyakit Adamantiades-Behçet (AB) merupakan kelainan inflamasi multisistemik yang tidak diketahui penyebabnya dengan manifestasi mukokutan tersering berupa ulkus berulang pada mulut dan genital. Penyakit ini terutama terjadi pada usia 20-30an. Lesi genital pada AB perlu dibedakan dengan ulkus genital akibat infeksi menular seksual termasuk herpes genitalis. Koinfeksi AB dan herpes genitalis jarang terjadi. Kami melaporkan kasus seorang laki-laki, usia 72 tahun, dengan keluhan luka di bibir dan kelamin yang nyeri sejak 1 minggu lalu, bersifat hilang-timbul selama 3 tahun terakhir. Riwayat nyeri sendi, sakit kepala berulang, serta kelainan kulit disangkal. Terdapat riwayat promiskuitas yang tinggi. Pada pemeriksaan fisik di regio labialis oris ditemukan ulkus multipel, diameter ±0,5-1 cm, tepi tidak teratur, dasar tertutup jaringan fibrin, dengan erosi dan krusta. Di regio glans penis ditemukan ulkus multipel, ukuran bervariasi ± 1x2x0,2cm, tepi tidak teratur, dasar tertutup pus dan jaringan nekrotik, terdapat punch out dan erosi. Tes patergi negatif. Pemeriksaan anti HSV-1 IgM (-), anti HSV-1 IgG (+), anti HSV-2 IgM dan IgG (+). Terapi asiklovir 5 x 200 mg/hari selama 5 hari, salep triamsinolon asetonid 2 kali oles, kompres terbuka NaCl 0,9% 3 x30 menit/hari, krim asam fusidat 2 kali oles, memberikan perbaikan klinis setelah 10 hari pengobatan. Prognosis quo ad vitam bonam, quo ad functionam, quo ad sanationam ad dubia. Simpulan: Pada kasus ini, diagnosis AB ditegakkan berdasarkan International Criteria for Behcet Disease yaitu ditemukannya ulkus berulang di mulut dan di genital, masing-masing mendapat nilai 2, sehingga nilai total ialah 4. Ditemukannya anti HSV-1 IgG, anti HSV-2 IgG dan IgM positif, menunjang diagnosis tambahan herpes genital (rekuren). Koinfeksi AB dengan herpes genital baru pertama kali dijumpai di Manado. Keadaan immunosenescence kemungkinan menjadi faktor pencetus terjadinya rekurensi herpes genital. Pasien sembuh dengan terapi simtomatis dan antivirus, meskipun kemungkinan rekurensi dapat terjadi lagi.Kata kunci: Adamantiades-Behcet, herpes genital, koinfeksi

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