Behçet disease (BD) is a systemic vasculitis with unknown etiology, characterized by relapsing episodes of oral and genital aphthous ulcers often associated with uveitis, skin lesions and frequent joint involvement. The central nervous system, gastrointestinal tract, and vessels are less frequently affected, but their involvement may result in life-threatening complications. This vasculitis can affect vessels of all sizes (small, medium and large caliber arteries, venules, veins) in multiple organs. Behçet’s disease is generally not a chronic persistent inflammatory disease, but rather a disease characterized by recurrent acute inflammatory flare-ups [1].

Central nervous system (CNS) involvement in BD is commonly referred to as Neuro-Behçet (NB) and is classified as a parenchymal, non-parenchymal or mixed syndrome [2] [3]. The parenchymal type has a worse prognosis. It is the more prevalent subtype and presents as brainstem, hemispheric, spinal, and meningoencephalitis manifestations. Non-parenchymal type includes cerebral venous sinus thrombosis (CVST) and arterial involvement [4].

The prevalence of NB is variable between 5% - 10% of patients with BD. It is more common in males with the age of onset of symptoms between 15 and 45 years [5].

The association of neurologic, pulmonary, and cardiovascular manifestations is very rare, and presents challenges with respect to disease management [6].

We report the case of a 25-year-old patient hospitalized for Neuro-Behçet complicated by pulmonary and cardiovascular involvement.

The diagnosis of Neuro-Behçet with cardiac and pulmonary thromboembolism was retained, according to the 2014 International Study Group Criteria for Behçet disease [7].

The medical treatment combined methylprednisolone (1 g/day) for 3 days, followed by oral prednisone 50 mg/day (1 mg/Kg/day), intravenous cyclophosphamide at a dose of 750 mg (15 mg/kg) every 4 weeks for a total of 6 cycles, followed by oral azathioprine, colchicine, and anticoagulation (heparin at curative doses relayed by antivitamin K).

The evolution of neurological symptoms under treatment was satisfactory with some resolution of motor deficits and persistence of spasticity and mild cerebellar symptoms. Cardiovascular and pulmonary involvement also progressed satisfactorily under anticoagulant therapy.

Neurological, cardiac, and vascular involvement in Behçet disease is very rare. The coexistence of manifestations in the same patient is exceptional. This makes management challenging, because of the very high risk of bleeding. The frequency of vascular involvement in Behçet disease is estimated to be between 2% - 46% in endemic regions. It is more common in men, and arterial lesions are less common than venous disease and its prevalence is about 1.5% - 3% worldwide [8].

This paper illustrates a case of Neuro-Behçet with systemic vascular involvement including arterial involvement in the form of a thrombosed aneurysm of the inferior lobar arteries, and cardiac involvement in the form of intracardiac thrombosis.

The mechanisms of thrombus formation are multiple: ischemia or rupture of endothelial cells, antiphospholipid antibodies found in 18% of cases or the presence of other prothrombotic plasma factors such as deficiency in protein C and or S [9], the increase in factor VIII, Leyden factor V homozygosity or prothrombin gene mutation [10].

After the first case by Budge et al. in 1977, around ten cases of intracardiac thrombosis with or without endomyocardial fibrosis were reported. Recently, 19.2% of cases of intracardiac thrombosis were reported in a study including 52 patients with cardiac involvement of Behçet’s disease [11]. Several factors have been implicated in the genesis of cardiac or vascular thrombosis, and include plasma prothrombotic factors, the presence of antibodies against enolase, and hyperhomocysteinemia [12].

According to the available data, 7% to 29% of patients with Behçet have vascular lesions [13]. During Behçet’s disease, aneurysms of the pulmonary arteries are considered exceptional, with a prevalence of 1.5% - 3% worldwide [8]. These aneurysms have been the subject of a few publications.

Clinically, these aneurysms are manifested by recurrent hemoptysis of low abundance, but in our case, it was a non-productive cough.

Arterial involvement occurs in 1% to 7% of patients with Behçet’s disease but may be at the forefront of the clinical picture causing life-threatening complications. The most commonly affected artery is the aorta, followed by the pulmonary artery, femoral artery, subclavian artery, popliteal artery, and common carotid artery [14].

Pulmonary artery aneurysms are rare, but most often represent the second aneurysmal location after the abdominal aorta. These aneurysmal lesions are usually multiple, bilateral and of proximal location involving the trunks and the lobar or segmental bronchi of the pulmonary arteries and involve a risk of hemoptysis and death due to arterial rupture [15].

Even rarer is the association of pulmonary arterial aneurysms with cardiac thrombosis. Consequently, the high risk of bleeding inherent in the aneurysm is potentiated by the administration of anticoagulants in the event of associated intracardiac thrombus, as is the case in our patient [16]. Several authors have reported the resolution of intracardiac thrombi after medical treatment combining: corticosteroids alone or associated with colchicine and/or immunosuppressants (azathioprine, cyclophosphamide, cyclosporine) and treatment with antivitamin K or aspirin [17] [18]. These data show the major role that vascular inflammation plays in the formation of the thrombus. With regard to vascular involvement, the surgical treatment of an aneurysm with all its risks in patients with Behçet’s disease frequently results in the recurrence of aneurysm.

Surgical treatment usually involves reconstruction using vascular grafts [19]. But it is often difficult and some specialists prefer to achieve only closure of the communication of the aneurysm. Neo-aneurysms have been observed at the vascular puncture sites and therefore exploration by arteriography should be avoided. Given the absence of a well-coded therapeutic approach concerning anticoagulation in the presence of such a very lethal pathological association due to its hemorrhagic and/or thrombotic risk, we opted for an initial treatment with heparin for better management of hemorrhagic accidents. The outcome of this treatment was favorable for our patient.

Neurological, cardiac, and vascular involvement in Behçet disease is very rare with higher mortality. The prognosis is unpredictable due to the occurrence of overwhelming hemoptysis. In our patient, pulmonary embolism and the right mobile atrial thrombus did not cause right heart failure and regressed totally under medical treatment.

Our sincere thanks to the Department of Neurology of the Mohammed VI University Hospital for the wonderful help from the staff, as well as to the patient for consenting to make this work possible.

The study was reviewed and approved on May 1, 2020 by the Ethics Committee of the Faculty of Medicine and Pharmacy of Marrakesh in accordance with the Declaration of Helsinki.

As approved by the ethics committee, the patient included in the study gave his verbal consent before inclusion in the study. Reference number not applicable.

All authors took part in the conceptualization of the Clinical presentation. Reine BIKOUTA and, Nissrine LOUHAB obtained and analyzed patient data and did the literature review; Reine BIKOUTA wrote the first draft. All authors read and approved the final manuscript.

The authors declare no conflicts of interest.

Bikouta Ouadika, R.J., Louhab, N., Benjilali, L. and Kissani, N. (2022) Pulmonary Arterial Thrombosed Aneurysm Associated with Intracardiac Thrombus and Pulmonary Embolism in a Patient with Neuro-Behçet’s Disease. Open Access Library Journal, 9: e8912. https://doi.org/10.4236/oalib.1108912

BD: Behçet Disease

CNS: Central Nervous System

NB: Neuro-Behçet

CVST: Cerebral Venous Sinus Thrombosis

MRI: Brain magnetic Resonance Imaging

CT: Computerized Tomography

TTE: Transthoracic Echocardiography