Behcet Disease Presenting With Cardiac and Pulmonary Masses

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

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Quality of Life in Patients With Behçet Disease and Its Relation With Clinical Symptoms and Disease Activity

Reumatología Clínica (English Edition) ◽

10.1016/j.reumae.2019.03.009 ◽

2021 ◽

Vol 17 (1) ◽

pp. 1-6

Author(s):

Alireza Khabbazi ◽

Vahideh Ebrahimzadeh Attari ◽

Mohammad Asghari Jafarabadi ◽

Aida Malek Mahdavi

Keyword(s):

Quality Of Life ◽

Disease Activity ◽

Clinical Symptoms ◽

Behçet Disease ◽

Behcet Disease

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335 - BEHÇET DISEASE PRESENTING WITH ACUTE PSYCHOSIS

International Psychogeriatrics ◽

10.1017/s1041610220002355 ◽

2020 ◽

Vol 32 (S1) ◽

pp. 94-94

Author(s):

A.M. Carvalheiro ◽

A.R. Fonseca ◽

J. Maia

Keyword(s):

Psychiatric Symptoms ◽

Retinal Vasculitis ◽

Posterior Uveitis ◽

Neurological Involvement ◽

Behçet Disease ◽

Acute Psychosis ◽

Behcet Disease ◽

Persecutory Delusions ◽

Starting Point ◽

History Of

ObjectivesUsing as a starting point a clinical case, the authors performed a literature review to clarify the relationship between Behçet disease and acute psychosis.MethodsAnalysis of the patient's clinical process and brief review of the latest available literature on the subject, published in PubMed/Medline databases.ResultsMale patient, 55 years old, brought to the emergency room by fever, headache, hetero-aggressive behavior, disinhibited behavior, mood swings, euphoria, persecutory delusions and insomnia, in the last 4 days. He had no insight into his illness. There was no personal or family history of psychiatric illness and toxicological habits were irrelevant. Due to the personal history of posterior uveitis with bilateral macular edema, retinal vasculitis, genital aphthosis, papulo-vesicular lesions and recurrent bipolar aphthosis, the hypothesis of neuro-behçet was raised.ConclusionsBehçet's disease can present with neurological involvement - neuro -behçet - and can manifest itself with several psychiatric symptoms (euphoria, lack of insight, disinhibited behavior, agitation or psychomotor retardation, persecutory delusions, obsessive thoughts, anxiety, depression, insomnia or memory changes). Fever and headache usually appear in the prodromal stage and can be signs of onset or recurrence of the disease. The prevalence of neuro-behçet ranges from 2 to 50% and usually occurs 1 to 10 years after the first symptoms of the disease. Since it appears as the first manifestation of the disease in only 3% of cases, it is difficult to diagnose. The literature suggests that symptoms are generally resistant to treatment with conventional psychotropic drugs and so it is an important cause of morbidity and mortality.”

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