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Aneurisma de la Aorta Abdominal , Síndrome de Behçet , Humanos , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Aneurisma de la Aorta Abdominal/diagnóstico por imagen , Aneurisma de la Aorta Abdominal/cirugía , Aneurisma de la Aorta Abdominal/complicaciones , Masculino , Angiografía por Tomografía Computarizada , AdultoRESUMEN
OBJECTIVE: People with Behçet's Disease, as many individuals with chronic diseases, often face depression, anxiety, poor quality of life and sexual problems. In this study, it was aimed to evaluate depression, anxiety, and sexual dysfuntions in people with Behcet's Disease. METHOD: A total of 100 participants, 50 patients (29 female) and 50 healthy volunteers (28 female), participated in the study. Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Female Sexual Function Index (FSFI), and International Index of Erectile Function (IIEF) were administered to the participants. RESULTS: Depression and sexual dysfunctions were significantly related with Behçet's Disease. In our study, all female participants with Behçet's Disease had problems in sexual functions. Erectile dysfunction was more frequent in participants with Behcet's. The results also showed that there is a significant relationship between depression and orgasmic function (p=0.004), sexual desire (p=0.028), sexual satisfaction (p=0.023), and general satisfaction (p=0.028). There was a significant difference between people with Behçet's Disease (10.54±6.45) and healthy group (7.36 ±6.13) in depression scores (p=0.009). Patients with systemic involvement and those with mucocutaneous involvement were found to be similar in terms of BDI and BAI scores (p>0.05). CONCLUSION: Behçet's Disease was found to be a risk factor for depression and sexual dysfunctions.
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Síndrome de Behçet , Disfunciones Sexuales Psicológicas , Humanos , Síndrome de Behçet/complicaciones , Síndrome de Behçet/psicología , Femenino , Adulto , Masculino , Disfunciones Sexuales Psicológicas/psicología , Disfunciones Sexuales Psicológicas/etiología , Estudios de Casos y Controles , Escalas de Valoración Psiquiátrica , Disfunciones Sexuales Fisiológicas/etiología , Disfunciones Sexuales Fisiológicas/psicología , Persona de Mediana Edad , Depresión/psicología , Adulto JovenRESUMEN
Behçet's disease is a systemic vasculitis of unknown origin. It mainly affects young men. Vascular involvement mainly affects the veins and may manifest as deep or superficial thrombosis. Arterial involvement is rare and serious. Arterial thrombosis or aneurysms/false aneurysms can be life threatening in case of rupture. All the arteries in the body can be affected, with widely varying frequencies. Involvement of the renal arteries is very rare. We report the case of a young patient followed for schizophrenia and known to have Behçet's disease, in whom a false aneurysm of the right renal artery was diagnosed during hematuria. He unfortunately refused any type of intervention.
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Aneurisma Falso , Síndrome de Behçet , Arteria Renal , Humanos , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamiento farmacológico , Masculino , Aneurisma Falso/etiología , Aneurisma Falso/diagnóstico por imagen , Aneurisma Falso/cirugía , Arteria Renal/diagnóstico por imagen , Adulto , Hematuria/etiología , Negativa del Paciente al Tratamiento , Angiografía por Tomografía ComputarizadaRESUMEN
BACKGROUND/AIMS: The association between inflammatory bowel disease (IBD) and gallstone and renal stone formation has been established. However, few studies have investigated this association in patients with intestinal Behçet's disease (BD). We aimed to examine the prevalence of gallstones and renal stones in patients with intestinal BD and identify potential risk factors. METHODS: We analyzed gallstone and renal stone occurrences in 553 patients diagnosed with intestinal BD who had undergone cross-sectional imaging examinations between March 2005 and April 2021 at the IBD Center, Severance Hospital, Seoul, South Korea. Logistic regression models were used to identify risk factors for gallstone and renal stone formation. RESULTS: Of 553 patients over a mean 12.1-year duration, 141 (25.4%) patients had gallstones and 35 (6.3%) had renal stones. In multivariate logistic regression analysis, disease duration > 19 years (OR 2.91, 95% CI 1.56-5.44, 0.002). No significant correlation 0.001), prior intestinal BD-related surgery (OR 2.29, 95% CI 1.42-3.68, p < 0.001), and disease activity index for intestinal BD scores ≥ 75 (OR 2.23, 95% CI 1.12-4.45, p = 0.022) were associated with increased gallstone occurrence. A positive correlation was observed between renal stones, disease duration > 19 years (OR 5.61, 95% CI 1.98-15.90, p = 0.001) and frequent hospitalization (> 3 times) (OR 3.29, 95% CI 1.52-7.13, p = 0.002). No significant correlation was observed between gallstone and renal stone occurrence. CONCLUSION: These findings contribute to greater understanding concerning gallstone and renal stone prevalence and associated risk factors in patients with intestinal BD.
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Síndrome de Behçet , Cálculos Biliares , Cálculos Renales , Humanos , Síndrome de Behçet/epidemiología , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Masculino , Femenino , Prevalencia , Factores de Riesgo , Cálculos Biliares/epidemiología , Cálculos Biliares/diagnóstico por imagen , Adulto , Persona de Mediana Edad , Cálculos Renales/epidemiología , Estudios Retrospectivos , República de Corea/epidemiología , Seúl/epidemiología , Adulto Joven , Estudios Transversales , Enfermedades Intestinales/epidemiologíaRESUMEN
To demonstrate the burden of sexual dysfunction (SD) among females with rheumatic diseases, we conducted a cross-sectional comparative study in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and Behçet's syndrome (BS) along with suitable healthy controls (HCs). Age-matched female patients with SSc (n = 50), SLE (n = 49), and BS (n = 54), along with 52 female HCs were included in this study between April and October, 2021. Sociodemographic features were recorded, and psychometric tests, i.e., female sexual function index (FSFI), Beck depression inventory (BDI), body cathexis scale, and marital adjustment test (MAT) were performed. Scale scores were compared, and binary logistic regression was used to identify predictors for SD in the whole group. The total FSFI and body cathexis scores among the patient groups were significantly lower than those of the HCs (p < 0.001). Depression was significantly more frequent in the patient groups. MAT scores did not differ significantly between the study groups. Patients with SSc had the worst scores in each psychometric index, including MAT. Decreased body cathexis score [OR 0.974, 95% CI (0.957-0.991), p = 0.003] and low MAT score [OR 0.937, 95% CI (0.896-0.980), p = 0.005], and being diagnosed with SSc [OR 6.6, 95% CI (1.975-22.498), p = 0.002], SLE [OR 2.7, 95% CI (0.998-7.753), p = 0.050], and BS [OR 2.8, 95% CI (1.100-7.359), p = 0.031], were identified as independent predictors for SD. Body cathexis seems to be the most important independent predictor for SD, and the burden of SD appears heavier in patients with SSc, probably due to poor body image satisfaction.
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Lupus Eritematoso Sistémico , Esclerodermia Sistémica , Disfunciones Sexuales Fisiológicas , Humanos , Femenino , Adulto , Estudios Transversales , Persona de Mediana Edad , Disfunciones Sexuales Fisiológicas/etiología , Disfunciones Sexuales Fisiológicas/psicología , Disfunciones Sexuales Fisiológicas/epidemiología , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/psicología , Lupus Eritematoso Sistémico/psicología , Lupus Eritematoso Sistémico/complicaciones , Depresión/psicología , Depresión/epidemiología , Depresión/etiología , Disfunciones Sexuales Psicológicas/psicología , Disfunciones Sexuales Psicológicas/etiología , Disfunciones Sexuales Psicológicas/epidemiología , Disfunciones Sexuales Psicológicas/diagnóstico , Enfermedades Reumáticas/psicología , Enfermedades Reumáticas/complicaciones , Síndrome de Behçet/complicaciones , Síndrome de Behçet/psicología , Estudios de Casos y Controles , PsicometríaRESUMEN
AIM: Behcet's disease (BD) is a systemic disorder characterized by vasculitis, resulting in thickened vascular walls that reduce elasticity and impair function. BD can involve the cardiovascular system in three ways: cardiac, arterial, and venous. In this study, our objective was to evaluate the efficacy of pulmonary arterial stiffness (PAS) and pulmonary pulse transit time (PPTT) measures in demonstrating right ventricular functions in asymptomatic BD patients. We aimed to objectively evaluate right ventricular function in patients with BD using four-dimensional echocardiography (4DE). METHOD: This study included 40 patients diagnosed with BD and 40 healthy subjects. Demographic, clinical, laboratory, and echocardiographic parameters were compared. In addition to standard transthoracic echocardiographic evaluation, right ventricle quantification (RVQ) by using the 4DE and 2D-speckle tracking echocardiography were performed. RESULTS: The sPAP, 4D RVQ, and right ventricular strain values exhibited significant differences between the BD and control groups. Right ventricular end-diastolic diameter (RVDD), right ventricular end-systolic diameter (RVSD), right atrium (RA) area, right ventricular myocardial performance index (RVMPI), and PAS were increased in BD patients compared to the control group. Right ventricular ejection fraction (RVEF), right ventricular fractional area change (RVFAC), tricuspid annular plane systolic excursion (TAPSE), Tricuspid S', and PPTT were decreased in BD patients compared to control subjects. PPTT correlated with right ventricular free wall strain (RV-FWS) and PAS. In a multivariate linear regression analysis, PAS and RVFAC were found to be independent predictors of RVFWS. In addition, RVFAC and TAPSE are independent predictors for PPTT. CONCLUSION: Patients with BD may have elevated pulmonary arterial stiffness (PAS) in correlation with decreased PPTT. To ascertain the prognosis for these individuals, right ventricular (RV) functions must be evaluated. Measurements of RVFAC and RVEF via 4DE and deformation imaging techniques may be more useful in identifying subclinical impairment of RV. Individuals with BD, PAS, and PPTT may suggest a link between early pulmonary vascular remodeling and RV subclinical impairment.
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Síndrome de Behçet , Disfunción Ventricular Derecha , Humanos , Síndrome de Behçet/fisiopatología , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico por imagen , Masculino , Femenino , Adulto , Disfunción Ventricular Derecha/fisiopatología , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/etiología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Reproducibilidad de los Resultados , Ecocardiografía/métodos , Arteria Pulmonar/fisiopatología , Arteria Pulmonar/diagnóstico por imagen , Función Ventricular Derecha/fisiología , Persona de Mediana Edad , Rigidez Vascular/fisiologíaRESUMEN
Cardiovascular involvement in Behçet's disease (BD) is considerably related to morbidity and mortality. However, the cardiovascular manifestation is sometimes difficult to distinguish from those of other causes. The suspicion of BD and proper treatment is pivotal in the management of BD. Histology demonstrates perivasculitis. Neutrophil seems to play an important role in the inflammation of BD. It is thought that inflammation causes venous thrombosis and arterial aneurysm. Characteristically, BD involves both arteries and veins of variable size in any region. Venous thrombosis needs immunosuppression, and inferior vena cava thrombosis and Budd-Chiari syndrome require intensive immunosuppressive therapy. Arterial involvement causes aneurysm which usually is treated by surgical or endovascular intervention with immunosuppression. Pulmonary artery aneurysm and cardiac involvement require multimodal managements.
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Síndrome de Behçet , Síndrome de Behçet/complicaciones , Síndrome de Behçet/patología , Humanos , Enfermedades Cardiovasculares/etiología , Arteria Pulmonar/patología , Aneurisma/etiología , Aneurisma/patología , Trombosis de la Vena/etiología , Trombosis de la Vena/patologíaRESUMEN
AIM: This study explores the connection between Behçet's disease (BD), characterized by persistent oral and genital ulcers alongside iritis, and periodontal disease. It examines the levels of tumor necrosis factor-α (TNF-α), interleukin-1ß (IL-1ß), and nitric oxide (NO) in gingival crevicular fluid (GCF) and saliva. METHODS: Forty Behçet's patients with gingivitis or periodontitis and 47 patients with either gingivitis or periodontitis but without BD were studied. Periodontal status was recorded with standard clinical indexes. GCF and saliva samples were obtained. NO, IL-1ß and TNF-α levels were analysed. Current Behçet's symptoms and medications usage were recorded. RESULTS: Mean salivary IL-1ß was elevated (p = .045), and mean NO level was decreased in BD patients with gingivitis compared to patients without BD (p = .000). In contrast, mean NO level in crevicular fluid was higher in Behçet's patients with periodontitis than in patients without BD (p = .009). Furthermore, among Behçet's patients, those with vascular involvement had lower salivary NO level compared to patients without vascular involvement (p = .000). CONCLUSIONS: Based on our findings, the elevated levels of IL-1ß in the saliva of Behçet's patients with gingivitis, along with the decreased NO level, indicate an altered inflammatory response in the oral cavity.
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Síndrome de Behçet , Líquido del Surco Gingival , Gingivitis , Interleucina-1beta , Óxido Nítrico , Saliva , Factor de Necrosis Tumoral alfa , Humanos , Síndrome de Behçet/complicaciones , Líquido del Surco Gingival/química , Interleucina-1beta/análisis , Interleucina-1beta/metabolismo , Masculino , Femenino , Saliva/química , Saliva/metabolismo , Gingivitis/metabolismo , Gingivitis/etiología , Adulto , Factor de Necrosis Tumoral alfa/análisis , Factor de Necrosis Tumoral alfa/metabolismo , Óxido Nítrico/metabolismo , Óxido Nítrico/análisis , Periodontitis/complicaciones , Periodontitis/metabolismo , Índice Periodontal , Persona de Mediana Edad , Bolsa Periodontal , Pérdida de la Inserción Periodontal , Adulto JovenRESUMEN
BACKGROUND: Neuro-Behcet's disease (NBD) is a variant of Behcet's disease (BD). To our knowledge, there have been no previous reports on concurrent NBD in breast cancer patients undergoing chemotherapy. CASE PRESENTATION: Our patient had a history of BD and was asymptomatic. She was diagnosed with human epidermal growth factor receptor 2-positive breast cancer by core needle biopsy and was administered neoadjuvant chemotherapy. After four courses, in addition to the aggravation of the existing adverse events, headache, fever, dysarthria, and muscle weakness in the upper left and lower extremities appeared. On admission, she was diagnosed with acute NBD, and steroid therapy was initiated. After her symptoms improved gradually, she was discharged. Then, she underwent mastectomy and axillary lymph node dissection for breast cancer. Trastuzumab and pertuzumab plus tamoxifen were administered postoperatively. Two years postoperatively, no recurrence of breast cancer and NBD was noted. CONCLUSION: When chemotherapy is administered to breast cancer patients with a history of BD, it is necessary to select chemotherapy with as few adverse events as possible and to continue with treatment while paying attention to the risk of NBD.
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Síndrome de Behçet , Neoplasias de la Mama , Terapia Neoadyuvante , Receptor ErbB-2 , Humanos , Femenino , Neoplasias de la Mama/tratamiento farmacológico , Síndrome de Behçet/complicaciones , Síndrome de Behçet/tratamiento farmacológico , Receptor ErbB-2/metabolismo , Mastectomía , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Trastuzumab/uso terapéutico , Trastuzumab/efectos adversos , Persona de Mediana Edad , Tamoxifeno/uso terapéutico , Tamoxifeno/efectos adversos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Anticuerpos Monoclonales Humanizados/efectos adversos , AdultoRESUMEN
BACKGROUND Pulmonary artery aneurysm (PAA), defined as a pathologic dilatation of the PA greater than 1.5-fold the normal diameter, is a rare complication of Behçet disease. It is due to a weakening of the vessel wall for a great vessels' vasculitis, often asymptomatic and incidentally diagnosed on imaging studies. However, if ignored, it can lead to life-threatening complications such as rupture and massive hemoptysis. We report the case of a giant fast-growing PAA in a young patient with a history of Behçet disease in which an inadequate follow-up and poor patient information could had led to life-threatening complications. CASE REPORT A 37-year-old man with a history of Behçet disease presented to our Emergency Department with hemoptysis due to a right inferior lobar artery aneurysm measuring 52×33 mm. The aneurysm was detected years before, measuring 18 mm, but the patient and physicians missed the subsequent follow-up. After several attempts at embolization, the multidisciplinary board suggested to proceed with surgical intervention. Surgery was performed with an extracorporeal circulation system kept on stand-by due to the high hemorrhagic risk. By opening the fissure, the dilatation of the inferior lobar artery was clearly identified up to the origin of the middle lobar branch. Thus, a lower-middle bilobectomy was performed after the introduction of a suction cannula in the aneurysm, which facilitated its emptying and the subsequent maneuvers. CONCLUSIONS PAA is a rare disease, generally treated with medical therapy or interventional procedures. However, giant and fast-growing aneurysms are more likely to entail complications and often required immediate treatment. In this case, primary surgical intervention with a pulmonary bilobectomy appeared mandatory to avoid life-threatening events.
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Aneurisma , Síndrome de Behçet , Arteria Pulmonar , Humanos , Síndrome de Behçet/complicaciones , Arteria Pulmonar/cirugía , Arteria Pulmonar/diagnóstico por imagen , Masculino , Adulto , Aneurisma/cirugía , Aneurisma/etiología , Hemoptisis/etiologíaRESUMEN
OBJECTIVES: To evaluate the microstructural integrity of brain white matter tracts in patients with Neuro-Behcet's syndrome (NBS) and Behcet's syndrome (BS) without neurological manifestations using diffusion tensor imaging (DTI) and to investigate potential utility of DTI as a surrogate biomarker of neurocognitive functioning and disease severity. METHODS: This cross-sectional study comprised 34 NBS patients and 32 BS patients without neurological involvement, identified based on the International Study Group of the Behcet's disease (ISGBD) and the International Consensus Recommendation (ICR) criteria, as well as 33 healthy controls. Cognitive functions, including attention, memory, language, abstraction, executive control, visuospatial skills, and sensorimotor performance were assessed using standardized questionnaires. DTI data were analyzed using tract-based spatial statistics (TBSS) and automated probabilistic tractography to investigate inter-group differences. Subsequently, correlations between tensor-derived parameters of white matter tracts, neurocognitive test scores, and disease severity measures were examined. RESULTS: DTI revealed decreased fractional anisotropy and increased radial diffusivity, mean diffusivity, and axial diffusivity in both supratentorial and infratentorial white matter in NBS patients, indicating widespread loss of microstructural integrity. Moreover, this loss of integrity was also observed in BS patients without neurological manifestations, albeit to a lesser extent. In NBS patients, certain white matter tracts, including cingulum bundle, were associated with poor cognitive performance across multiple domains and disease severity. DISCUSSION: DTI findings might potentially serve as a neuroimaging marker to predict the extent of neurocognitive impairment and disease severity associated with central nervous system involvement in BS.
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Síndrome de Behçet , Disfunción Cognitiva , Imagen de Difusión Tensora , Índice de Severidad de la Enfermedad , Sustancia Blanca , Humanos , Síndrome de Behçet/diagnóstico por imagen , Síndrome de Behçet/complicaciones , Síndrome de Behçet/patología , Masculino , Femenino , Adulto , Estudios Transversales , Sustancia Blanca/diagnóstico por imagen , Sustancia Blanca/patología , Disfunción Cognitiva/diagnóstico por imagen , Disfunción Cognitiva/etiología , Persona de Mediana Edad , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Pruebas NeuropsicológicasRESUMEN
ABSTRACT: Cerebral venous thrombosis (CVT) is a rare stroke with multiple risk factors. One rare risk factor is Behçet's disease (BD). Out of around 3000 cases at our center in the past 10 years, two cases of BD with CVT were seen. Herein, we report on their clinical symptoms, course, and management. Case 1 was a 18-year-old girl with a history of recurrent skin lesions presenting with encephalopathy syndrome due to CVT, requiring decompression. Despite our best efforts, she developed complications and expired due to sepsis. Case 2 was a 22-year-old male with raised intracranial pressure syndrome and a history of recurrent orogenital ulcers. His evaluation showed retinal vasculitis, papilledema, and bilateral lateral rectus palsy. Both had CVT on neuroimaging and had positivity for human leukocyte antigen-B51. Case 2 responded to the anticoagulation and immunomodulation. Risk factor identification is essential in managing CVT, and planned evaluation (clinical or investigations) plays an important role in identifying rare causes that need specific treatment.
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Anticoagulantes , Síndrome de Behçet , Trombosis Intracraneal , Trombosis de la Vena , Humanos , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamiento farmacológico , Masculino , Adolescente , Femenino , Trombosis de la Vena/etiología , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/tratamiento farmacológico , Trombosis de la Vena/diagnóstico por imagen , Trombosis Intracraneal/diagnóstico por imagen , Trombosis Intracraneal/etiología , Trombosis Intracraneal/diagnóstico , Anticoagulantes/uso terapéutico , Adulto Joven , Imagen por Resonancia MagnéticaRESUMEN
The patient 1, a 13-year-old boy, was admitted due to "recurrent oral ulcers for 3 years, abdominal pain for 8 months, and perianal ulcers for 10 days"; The patient 2, a 3-year-old boy, was admitted due to "recurrent abdominal pain, diarrhea, and fever for over 3 months". Genetic testing of both patients revealed "deficiency in ELF4, X-linked" (DEX), and the patients were diagnosed with Behcet's disease-like syndrome due to deficiency in ELF4, accordingly. The patient 1 was successively given intravenous methylprednisolone pulses and oral prednisone and mesalazine for symptomatic treatment. The patient 2 was successively treated with corticosteroids combined with enteral nutrition, as well as oral mercaptopurine. Subsequently, both patients showed improvements in symptoms and were discharged.
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Síndrome de Behçet , Humanos , Masculino , Síndrome de Behçet/genética , Síndrome de Behçet/complicaciones , Preescolar , AdolescenteAsunto(s)
Aneurisma , Arteria Axilar , Síndrome de Behçet , Microaneurisma , Humanos , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamiento farmacológico , Arteria Axilar/diagnóstico por imagen , Aneurisma/diagnóstico por imagen , Aneurisma/etiología , Resultado del Tratamiento , Masculino , Microaneurisma/etiología , Microaneurisma/diagnóstico , Adulto , Angiografía por Tomografía Computarizada , Embolización TerapéuticaAsunto(s)
Artritis Reumatoide , Azetidinas , Síndrome de Behçet , Purinas , Piodermia Gangrenosa , Pirazoles , Sulfonamidas , Humanos , Pirazoles/uso terapéutico , Azetidinas/uso terapéutico , Sulfonamidas/uso terapéutico , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamiento farmacológico , Purinas/uso terapéutico , Artritis Reumatoide/complicaciones , Artritis Reumatoide/tratamiento farmacológico , Piodermia Gangrenosa/tratamiento farmacológico , Piodermia Gangrenosa/etiología , Piodermia Gangrenosa/diagnóstico , Resultado del Tratamiento , Femenino , Masculino , Persona de Mediana EdadRESUMEN
Background/aim: Our primary aim was to investigate the effects of concomitant celiac disease (CD) on the clinical characteristics of Behçet's syndrome (BS) patients. Materials and method: The study was a retrospective, nationwide, multicenter study. Turkish Ministry of Health National Electronic Database (e-Nabiz) is used under Health Ministry's supervision to extract the subject's data. Statistical analysis: Statistical analyses were made by the Statistical Package for Social Sciences (SPSS) software version 20 (IBM Corp., Armonk, New York). Continuous variables were presented by mean ± standard derivation (SD) or median (min-max) according to normality and compared by student-t test. A binary logistic regression analysis was performed to further investigating the relation between having a concomitant CD with each BD manifestation and comorbidity, frequencies of which were detected to be significantly different in the student-test. Results: A total of 84,241 patients diagnosed with BS were analyzed, and CD was identified in 175 (0.21 %) patients. The group with CD had a mean age of 41.30 ± 13.69 which was significantly younger. the prevalence of females was significantly higher (71.4%). The mean age of first admission for BS was also significantly younger in the group with CD (36.64 ± 13.28). BS patients with CD had a significantly higher prevalence of inflammatory bowel disease (27.2% vs. 7.3%, p < 0.001). When comorbid conditions were investigated depression (35.4% vs. 23.3%, p < 0.001), migraine (7.4 % vs. 2.6%, p < 0.001), fibromyalgia (10.9% vs. 4.5%, p < 0.001) and osteoporosis (12.6% vs. 6.6%, p = 0.001) were significantly more frequent in BS patients with CD. Conclusion: Our results suggest coexistence of CD in BS patients is related to female dominance and probably to an earlier disease onset. Several CD-related comorbidities as well as inflammatory bowel disease were more frequent in the CD group which implied an increased overall disease burden.
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Síndrome de Behçet , Enfermedad Celíaca , Humanos , Síndrome de Behçet/epidemiología , Síndrome de Behçet/complicaciones , Enfermedad Celíaca/epidemiología , Enfermedad Celíaca/complicaciones , Femenino , Masculino , Adulto , Turquía/epidemiología , Estudios Retrospectivos , Persona de Mediana Edad , Bases de Datos Factuales , Comorbilidad , Prevalencia , Adulto JovenRESUMEN
AIM: Pulmonary artery involvement is a severe complication of Behcet's disease (BD). Although venous thrombosis is common in BD, pulmonary embolism is considered to be rare because the inflammatory nature makes the thrombi strongly adherent to the venous walls. This study aimed to define the radiological characteristics of pulmonary artery thrombosis (PAT) on computed tomography (CT) imaging in BD patients. METHODS: We retrospectively evaluated 165 BD patients with vascular involvement. Among the patients with venous involvement (n = 146), we identified 65 patients who had undergone thorax CT imaging previously. Fourteen patients who were diagnosed with PAT were included in the study. Expert radiologists re-evaluated the patients' initial and control thorax CT scans, classified the PAT as acute or chronic based on their radiological features. RESULTS: The patients' median age was 35 (min-max: 15-60) years at the time of the initial CT scan, and nine were male. Twelve (85.7%) patients were symptomatic at the time of CT evaluation. Upon re-evaluating the thorax CTs, acute PAT was diagnosed in six (42.8%); chronic PAT was detected in eight (57.1%) patients. Two patients with chronic PAT also had acute PAT. Pulmonary artery aneurysms were present in three (21.4%) patients, and intracardiac thrombus was found in three (21.4%) patients. CONCLUSION: A significant number of BD patients with venous involvement had radiological findings consistent with acute PAT potentially due to pulmonary emboli in this study. The clinical importance of these lesions has to be defined with future studies.
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Síndrome de Behçet , Angiografía por Tomografía Computarizada , Valor Predictivo de las Pruebas , Arteria Pulmonar , Trombosis , Humanos , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico por imagen , Síndrome de Behçet/diagnóstico , Masculino , Femenino , Arteria Pulmonar/diagnóstico por imagen , Adulto , Estudios Retrospectivos , Persona de Mediana Edad , Adulto Joven , Adolescente , Trombosis/diagnóstico por imagen , Trombosis/etiología , Enfermedad Crónica , Enfermedad Aguda , Embolia Pulmonar/diagnóstico por imagen , Embolia Pulmonar/etiologíaRESUMEN
Behçet's disease (BD) is a multifaceted autoimmune disorder affecting multiple organ systems. Vascular complications, such as venous thromboembolism (VTE), are highly prevalent, affecting around 50% of individuals diagnosed with BD. This study aimed to identify potential biomarkers for VTE in BD patients. Three microarray datasets (GSE209567, GSE48000, GSE19151) were retrieved for analysis. Differentially expressed genes (DEGs) associated with VTE in BD were identified using the Limma package and weighted gene co-expression network analysis (WGCNA). Subsequently, potential diagnostic genes were explored through protein-protein interaction (PPI) network analysis and machine learning algorithms. A receiver operating characteristic (ROC) curve and a nomogram were constructed to evaluate the diagnostic performance for VTE in BD patients. Furthermore, immune cell infiltration analyses and single-sample gene set enrichment analysis (ssGSEA) were performed to investigate potential underlying mechanisms. Finally, the efficacy of listed drugs was assessed based on the identified signature genes. The limma package and WGCNA identified 117 DEGs related to VTE in BD. A PPI network analysis then selected 23 candidate hub genes. Four DEGs (E2F1, GATA3, HDAC5, and MSH2) were identified by intersecting gene sets from three machine learning algorithms. ROC analysis and nomogram construction demonstrated high diagnostic accuracy for these four genes (AUC: 0.816, 95% CI: 0.723-0.909). Immune cell infiltration analysis revealed a positive correlation between dysregulated immune cells and the four hub genes. ssGSEA provided insights into potential mechanisms underlying VTE development and progression in BD patients. Additionally, therapeutic agent screening identified potential drugs targeting the four hub genes. This study employed a systematic approach to identify four potential hub genes (E2F1, GATA3, HDAC5, and MSH2) and construct a nomogram for VTE diagnosis in BD. Immune cell infiltration analysis revealed dysregulation, suggesting potential macrophage involvement in VTE development. ssGSEA provided insights into potential mechanisms underlying BD-induced VTE, and potential therapeutic agents were identified.
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Síndrome de Behçet , Biomarcadores , Biología Computacional , Perfilación de la Expresión Génica , Mapas de Interacción de Proteínas , Humanos , Síndrome de Behçet/genética , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Biología Computacional/métodos , Mapas de Interacción de Proteínas/genética , Biomarcadores/sangre , Redes Reguladoras de Genes , Trombosis de la Vena/genética , Trombosis de la Vena/etiología , Trombosis de la Vena/diagnóstico , Tromboembolia Venosa/genética , Tromboembolia Venosa/etiología , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/sangre , Factor de Transcripción GATA3/genética , Curva ROC , Histona Desacetilasas/genética , Aprendizaje AutomáticoRESUMEN
BACKGROUND Vascular Behçet's disease (VBD) is a rare but potentially life-threatening subtype of Behçet's disease that is characterized by multisystemic vasculitis. It primarily affects males with ancestry traced back to regions along the ancient Silk Road. Both arteries and veins, regardless of size, may exhibit complications, including aneurysmal degeneration or occlusion. While venous involvement is observed in two-thirds of VBD cases, arterial complications are notably the most severe and lethal. Arterial aneurysmal degeneration is more common than occlusive complications, with larger arteries being predominantly affected in VBD. Data regarding isolated small-vessel arterial occlusive disease in VBD are limited. Given the rarity of this presentation in this patient population, it becomes mandatory to thoroughly evaluate such patients to differentiate small-vessel vasculitis from other similar diseases, such as Raynaud's phenomenon, which has a different etiology and management and generally has a more benign course. Here, we delineate the concept of isolated small-vessel vasculitis as a cause of blue toe syndrome in patients with VBD. CASE REPORT This report describes a distinctive case of vascular Behçet's disease in a 51-year-old man who initially exhibited unilateral blue toe syndrome, which swiftly progressed to dry gangrene of the toes. Despite reports of large-vessel involvement, there is a paucity of data on isolated small-vessel vasculitis-induced digital ischemia in VBD. CONCLUSIONS This atypical case underscores the necessity of clinical discernment in differentiating inflammatory microvascular occlusive disease from vasospastic Raynaud's syndrome, both of which can complicate Behçet's disease.