A 30-year-old female Behçet’s disease patient with recurrent pleural and pericardial effusion and elevated adenosine deaminase levels: case report

Abstract

Behçet's disease is a systemic disease which may involve various organs. We describe a case of a patient diagnosed as pleuropericardial involvement of Behçet's disease. A 30-year-old woman visited our clinic presented with left pleuritic chest pain for s days. She had been diagnosed as Behçet's disease and admitted to our clinic due to pericardial and pleural effusion repeatedly in past two years. In the previous studies, effusion analysis revealed to be lympho-dominant exudate with high adenosine deaminase level. Acid-fast bacilli (AFB) culture and polymerase chain reaction (PCR) for mycobacterial tuberculosis (M.TB) were negative in the pericardial tissue, and pathologic finding showed mild endothelitis with micro-thrombi formation in the lumen. The patient had been treated with antituberculous medication for a year. In the current admission, chest computed tomography (CT) again showed left pleural effusion without other significant lesion. Pleural fluid analysis was similar with the previous study. Video-assisted thoracoscopic pleural biopsy was performed to obtain the definite diagnosis. Pathology confirmed the diagnosis as pleuropericardial involvement of Behçet's disease, and we treated the patient with oral steroid in the out-patient department. Pleuropericardial involvement of Behçet's disease may mimic TB pleurisy or pericarditis due to high adenosine deaminase (ADA) level in effusion analysis. Clinicians should keep in mind that Behçet's disease may manifest as pleural or pericardial effusion, and pathologic confirmation could be helpful for the definite diagnosis.Keywords: Pleural effusion; pericardial effusion; Behçet's syndrome; adenosine deaminase Submitted Jan 06, 2016. Accepted for publication May 11, 2016May 11, . doi: 10.21037/jtd.2016 View this article at: http://dx.doi.org/10.21037/jtd.2016.05.88 J Thorac Dis 2016;8(7):E547-E551 jtd.amegroups.com syndrome or pulmonary infarction. A few case reports have indicated pleural effusion due to primary vasculitis of the pleura (6). Cardiovascular involvement, including coronary artery disease, pericarditis, myocarditis, endocarditis, arrhythmia and valvular dysfunction, have been reported in <10% of patients with Behçet's disease (9-11). Pericardial involvement, a common cardiac manifestation with up to 40% cardiac involvement, may be associated with vasculitis of the pericardium or SVC thrombosis (6,10).The main pathologic characteristic of Behçet's disease is nonspecific vasculitis of small-to-large-sized vessels in both the systemic and pulmonary circulation, with perivascular infiltration of lymphocytes and mononuclear cells (12). Vasculitis mostly affects veins and accounts for approximately 85% of the vascular involvement (3). Endothelial cell swelling, fibrinoid necrosis and thrombus formation within the vessel lumen are common in inflammation foci (5,8).Anti-inflammatory and immunosuppressive drugs represent the mainstay treatments for Behçet's disease (7,8). However, the choice of treatment varies depending on the type of organ ...

Rare paediatric cardiopulmonary presentation of Behcet’s disease

“…He had a significant effusion with cardiac tamponade and multiple small emboli, but imaging did not show a large thrombus (figure 4). There is a reported case of a 30-year-old woman with recurrent pleuropericardial effusions and underlying Behcet’s disease 11. It is likely in this case and with our patient that the underlying panserositis was the cause of effusions and not a single significant thrombus.…”

Homem com úlceras orais, febre e hiperemia conjuntival

“…Derrame pleural é incomum e geralmente secundário à síndrome de veia cava superior, infarto pulmonar ou à doença infecciosa após início do tratamento imunossupressor. Apenas 1,5% dos pacientes com envolvimento pulmonar apresentam aneurismas de artéria pulmonar, com ou sem trombose, condição esta de extrema gravidade clínica e que deve ser ativamente investigada 3,[11][12][13][14] Neste contexto, foram realizadas angiotomografias de tórax e abdome, que não evidenciaram falhas de enchimento arterial ou venoso nos segmentos estudados, incluindo veia cava superior e inferior. Para complementar investigação etiológica do edema de face do paciente, foi solicitado ecodoppler venoso colorido, o que revelou trombose venosa na veia jugular interna direita, já com recanalização, além de tromboflebite na veia braquial esquerda, uma das quatro manifestações clínicas mais características e último critério utilizado para o diagnóstico de DB.…”

“…O acometimento concomitante cardíaco e pulmonar pela DB é incomum e geralmente está associado a um pior prognóstico, caso em que se indica tratamento com imunossupressão. 3,4,[11][12][13]16 Para tanto, infecções latentes devem ser pesquisadas e adequadamente tratadas, especialmente em locais onde a prevalência é alta, como no Rio de Janeiro. O aneurisma de artéria pulmonar deve ser pesquisado em todos os pacientes com acometimento pulmonar, tendo em vista a alta mortalidade associada.…”

“…1,[3][4][5]11,13,15 Após estabilização hemodinâmica do paciente e investigação diagnóstica, optamos pelo início de terapia imunossupressora com ciclofosfamida devido à gravidade do quadro. O teste tuberculínico (PPD) realizado antes do início da pulsoterapia foi forte reator, e iniciou-se tratamento para tuberculose latente com isoniazida.…”