scholarly journals Pulmonary artery thrombosis as the first presentation of Behçet’s syndrome: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ziyad Alakkas ◽  
Waad Kazi ◽  
Mohamed Mattar ◽  
Eman Abdul Wahhab Salem ◽  
Naglaa Fawzy Seleem
Keyword(s):  
Pulmonary Artery ◽  
Morbidity And Mortality ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Artery Thrombosis ◽  
Behcet’S Syndrome ◽  
Genital Ulcers ◽  
Behcet's Syndrome

Abstract Background Behçet’s syndrome is a type of systemic chronic vasculitis of unknown etiology, frequently characterized by recurrent oral and genital ulcers and uveitis. It is less commonly characterized by arthritis and skin, vascular, and gastrointestinal involvements. Behçet’s syndrome affects various sizes of vessels by perivascular infiltration and vasculitis. Unlike other classic types of vasculitis, Behçet’s syndrome patients can present with both arterial and venous involvement. Although vascular Behçet’s syndrome is found in only around 15% of Behçet’s syndrome patients, it is the major cause of morbidity and mortality among them. Furthermore, although deep venous thrombosis has high incidence in Behçet’s syndrome patients, pulmonary artery thrombosis is an uncommon complication. Combining the findings of this and previous case reports of pulmonary artery thrombosis in Behçet’s syndrome patients, we sought to determine the best treatment options for pulmonary artery thrombosis in Behçet’s syndrome patients. Case presentation We present the case of a 22-year-old Arabian male who was admitted to an emergency department with acute chest pain, dyspnea, and hemoptysis for 2 weeks. He gave a long history of recurrent oral and genital ulcers for the last 4 months but without seeking medical advice. Spiral computed tomography showed arterial filling defects with a pulmonary nodule for which the presence of a pulmonary artery aneurysm ruled out. The lung perfusion scan showed multiple pulmonary perfusion defects. After excluding common infectious diseases such as tuberculosis and brucellosis, a diagnosis of Behçet’s syndrome with pulmonary artery thrombosis was made. Steroids with enoxaparin were initiated. The patient was discharged later on prednisolone (tapering dose) with adalimumab and apixaban. He was on regular follow-up for the next 9 months. Conclusions Vascular involvement in Behçet’s syndrome is a major contributor to morbidity and mortality of Behçet’s syndrome patients. Consequently, early detection of vascular involvement has a major impact on the prognosis of patients with Behçet’s syndrome.

Behçet’s Syndrome Complicated with Pulmonary Artery Thrombosis: Response to Tocilizumab Treatment

2021 ◽  
Author(s):  
Didem Sahin Eroglu ◽  
Anil Colaklar ◽  
Alparslan Ceran ◽  
Serdar Sezer ◽  
Murat Torgutalp ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Artery Thrombosis ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

ZusammenfassungDie Beteiligung der Lungenarterie ist eine seltene, aber tödliche Komplikation des Morbus Behçets (MB). Hier berichten wir über einen männlichen MB-Patienten mit zwei vaskulären Komplikationen, nämlich dem Budd-Chiari-Syndrom und der Pulmonalarterienthrombose. Der Patient litt unter einer rezidivierenden Lungenarterienthrombose, die gegen mehrere immunsuppressive Medikamente, einschließlich hochdosierter Glukokortikoide und dem Anti-TNF-Mittel Infliximab, refraktär war. Infolge mehrerer Rezidive wurde er effektiv mit Tocilizumab behandelt, wodurch auch eine vollständige Rekanalisation der thrombosierten Arterien erreicht wurde.

P01-228-Behcet's syndrome comorbid with bipolar disorder: A case report

2011 ◽  
Vol 26 (S2) ◽  
pp. 229-229
Author(s):  
F. Maner ◽  
Ö. Şahmelikoğlu ◽  
Ö. Hısım ◽  
H. Özhan ◽  
H. Sarıahmetoğlu ◽  
...  
Keyword(s):  
Bipolar Disorder ◽  
Manic Episode ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Behçet’S Syndrome ◽  
Psychomotor Activity ◽  
Chronic Inflammatory Disorder ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

IntroductionBehcet's Syndrome is a chronic inflammatory disorder of unknown etiology, characterized by aphthous lesions and recurrent ulceration of the mouth, genitals and uveitis.ObjectivesThe central nervous system is involved in about 20% of cases.AimsOnly few reports deal with affective symptoms associated with Behcet's syndrome.MethodsWe report a case of a 43 year old male with Neuro-Behcet's Syndrome that presents with a psychotic manic attack. He developed Behcet's Syndrome at the age of 23, with recurrent uveitis and aphthous lesions in the mouth, painful ulcers in the genitalia and erythema nodosum. HLA-B 5 was positive.ResultsHe was treated with azothioprine 150 mg/day for 13 years and prednole 100 mg/day during uveitis attacts for a week. At the age of 37 a sudden occurrence of right hemiparesia due to cerebrovascular accidence salicylic acid 100mg/day, siclosporine 150 mg/day, piracetame 1600mg/day were administered. He presented to psychiatry clinic in manic episode with euphoric mood, psychomotor agitation, talkativeness, decreased need for sleep, excessive buying and he had an unrealistical thought that he was a player of a famous soccer team. He was diagnosed as bipolar I disorder, according to DSM-IV. This was the patient's first admission and the symptoms which were continuing for 6 years exaggerated during uveitis attacks.Psychiatric examination releaved that increaced psychomotor activity, hypomaniac affect, amount and affect speed of speech affect, increased associations, grandiose delusions.ConclusionThere are a few reports dealing with bipolar disorder as an entity related to Behcet's syndrome.

scholarly journals Effects of corticosteroid and chlorambucil on multiple pulmonary artery aneurysms in Behcet's syndrome: A case repor

1995 ◽  
Vol 10 (6) ◽  
pp. 470 ◽  
Author(s):  
Jae Yong Park ◽  
Jun Goo Park ◽  
Jun Hee Won ◽  
Jong Myung Lee ◽  
Nung Soo Kim ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Pulmonary Artery Aneurysms ◽  
Behcet's Syndrome

scholarly journals Modulation of gut microbiota through nutritional interventions in Behçet's syndrome patients (the MAMBA study): study protocol for a randomized controlled trial

2019 ◽  
Author(s):  
Giuditta PAGLIAI ◽  
Monica DINU ◽  
Claudia FIORILLO ◽  
Matteo BECATTI ◽  
Silvia TURRONI ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Mediterranean Diet ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Processed Meat ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Wide Range ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Background Behçet's syndrome (BS) is a systemic inflammatory disorder of unknown etiology, characterized by a wide range of potential clinical manifestations. Recent evidences suggest that the gut microbiota (GM) in BS shows low biodiversity with a significant depletion in butyrate producers. The aim of the present project is to investigate whether a dietary intervention could ameliorate the clinical manifestations and modulate the GM of patients with BS.Methods This is a randomized, open, cross-over study involving 90 BS patients who will be randomized to follow a 3-months dietary profile with either: lacto-ovo-vegetarian diet (VD), Mediterranean diet (MD) or Mediterranean diet supplemented with butyrate (MD-Bt). The VD will contain inulin and resistant starch-rich foods, eggs and dairy, in addition to plant-based food, but will not contain meat, poultry or fish. The MD will contain all food categories and will provide 2 portions per week of fish and 3 portions per week of fresh and processed meat. The MD-Bt will be similar to the MD but supplemented with 1.8 g/day of oral butyrate. The three different dietary patterns will be isocaloric and related to subject’s nutritional requirements. Anthropometric measurements, body composition, blood and fecal samples will be obtained from each participant at the beginning and at the end of each intervention phase. The primary outcomes will be represented by the change from baseline of the BS gastrointestinal and systemic symptoms. Changes from baseline of GM composition, SCFA production, inflammatory and antioxidant profile will be considered as secondary outcomes.Discussion BS is a rare disease, and, actually, not all the available treatments are target therapies. A supportive treatment based on dietary and lifestyle issues, able to restore immune system homeostasis, could have a high impact on costs sustainability for the treatment of such a chronic and disabling inflammatory condition.

scholarly journals AB0532 CORRELATION WITH THE FREQUENCY OF DISEASE RELAPSES DURING THE FIRST 3 YEARS FROM THE DIAGNOSIS AND DISEASE OUTCOMES IN BEHCET’S SYNDROME

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1563.2-1563
Author(s):  
R. Talarico ◽  
A. Figliomeni ◽  
L. Mione ◽  
A. Parma ◽  
E. Cioffi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Disease Onset ◽  
Disease Outcome ◽  
Vascular Involvement ◽  
Joint Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Disease Outcomes ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Background:Beside the organ involvement, a number of demographic factors could considerably influence the long-term and short-term outcomes of Behçet’s syndrome (BS): age at disease onset, duration of disease, gender and sex. Younger men patients are more suitable to have a more severe disease, due to an increased frequency both of morbidity and mortality, related to ocular,vascular and neurological involvementObjectives:The primary aims of the study were to evaluate disease activity in a cohort of BS patients consecutively followed in a BS clinic of a tertiary centre and to explore whether there is a correlation between frequency of relapses in the first 3 years of diseases and disease outcomes.Methods:One-hundred and sixty-five patients (91 males and 74 females; mean age 39±9 years, mean disease duration 9±5) with a diagnosis of BS according to the ISG criteria were studied. Disease activity has been evaluated by BDCAF and patients were also categorized in major or minor involvement of BS according or not to the presence of ocular, neurological and vascular involvement in the course of disease The numbers of relapses in the first 3 years from diagnosis were correlated with disease outcome and damage.Results:At time of the evaluation, 47% of BS patients presented an active disease; 69 patients presented muco-cutaneous involvement, 39 ocular disease, 21 joint involvement, 12 neurological impairment and 9 gastro-enteric involvement. Seventy-nine percent of patients presented in the course of the disease a severe BS involvement and the majority was represented by patients characterised by a more frequent relapse in the first 3 years of disease (M/F: 65/48, mean age 43±3 years). Those patients who experienced a more higher number of relapse in the first 3 years compared to the others were also characterised by poor disease outcomes and worse prognosis over time and this correlation was independent by the therapies taken.Conclusion:The high frequency of relapses during the first three years from diagnosis may be considered an important prognostic factor for disease outcome in BS patients, therefore could be taken into account as a useful element to tailor the management, not only according to the type and severity of symptoms and epidemiological factors.Acknowledgments:noneDisclosure of Interests:None declared

scholarly journals Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Jun Zou ◽  
Jian-feng Luo ◽  
Yan Shen ◽  
Jian-fei Cai ◽  
Jian-long Guan
Keyword(s):  
Central Nervous System ◽  
Cluster Analysis ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
System Involvement ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Introduction Behcet’s syndrome (BS) is a complex, heterogeneous disorder. However, classification of its subgroups is still debated. The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements. Methods This was a cross-sectional study of BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020. We calculated relative risks (RRs) of clinical variables according to sex. Moreover, we conducted a hierarchical cluster analysis applied according to eighteen variables to determine subgroups of patients. Results A total of 860 BS patients were included. Male sex was associated with ocular involvement (RR 2.32, 95% CI 1.67, 3.22, P < 0.0001), vascular involvement (RR 2.00, 95% CI 1.23, 3.23, P = 0.004), cardiac lesion (RR 5.46, 95% CI 2.33, 12.77, P < 0.0001), and central nervous system involvement (RR 2.95, 95% CI 1.07, 6.78, P = 0.007) and was negatively associated with genital ulcers (RR 0.84, 95% CI 0.79, 0.91, P < 0.0001). Five clusters (C1–C5) were observed. C1 (n = 307) showed the skin and mucosa type. In C2 (n = 124), all had articular involvement, barely having major organ involvement except for 18 cases with intestinal lesions. In C3 (n = 156), the gastrointestinal type, 144 patients presented with intestinal involvement, and 36 patients with esophageal ulcers. In C4 (n = 142), all subjects presented with uveitis. C5 (n = 131) consisted of 44 patients with cardiac lesions, 58 with vascular involvement, and 26 cases having central nervous system involvement. Conclusion Our analysis confirmed sex differences in phenotypes of BS. Cluster analysis identified gastrointestinal, uveitis, and cardiovascular involvement cluster separately in different subsets, which represents the most commonly involved organs. Further research is required to replicate and clarify the patterns of phenotype in BS.

scholarly journals Modulation of gut microbiota through nutritional interventions in Behçet's syndrome patients (the MAMBA study): study protocol for a randomized controlled trial

2020 ◽  
Author(s):  
Giuditta PAGLIAI ◽  
Monica DINU ◽  
Claudia FIORILLO ◽  
Matteo BECATTI ◽  
Silvia TURRONI ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Mediterranean Diet ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Processed Meat ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Wide Range ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Background: Behçet's syndrome (BS) is a systemic inflammatory disorder of unknown etiology, characterized by a wide range of potential clinical manifestations. Recent evidences suggest that the gut microbiota (GM) in BS shows low biodiversity with a significant depletion in butyrate producers. The aim of the present project is to investigate whether a dietary intervention could ameliorate the clinical manifestations and modulate the GM of individuals with BS. Methods: This is a randomized, open, cross-over study involving 90 BS individuals who will be randomized to follow a 3-months dietary profile with either: lacto-ovo-vegetarian diet (VD), Mediterranean diet (MD) or Mediterranean diet supplemented with butyrate (MD-Bt). The VD will contain inulin and resistant starch-rich foods, eggs and dairy, in addition to plant-based food, but will not contain meat, poultry or fish. The MD will contain all food categories and will provide 2 portions per week of fish and 3 portions per week of fresh and processed meat. The MD-Bt will be similar to the MD but supplemented with 1.8 g/day of oral butyrate. The three different dietary patterns will be isocaloric and related to participants’ nutritional requirements. Anthropometric measurements, body composition, blood and fecal samples will be obtained from each participant at the beginning and at the end of each intervention phase. The primary outcomes will be represented by the change from baseline of the BS gastrointestinal and systemic symptoms. Changes from baseline of GM composition, SCFA production, inflammatory and antioxidant profile will be considered as secondary outcomes. Discussion: BS is a rare disease, and, actually, not all the available treatments are target therapies. A supportive treatment based on dietary and lifestyle issues, able to restore immune system homeostasis, could have a high impact on costs sustainability for the treatment of such a chronic and disabling inflammatory condition.

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