Pulmonary Arterial Thrombosed Aneurysm Associated with Intracardiac Thrombus and Pulmonary Embolism in a Patient with Neuro-Behçet’s Disease ()
1. Introduction
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.
2. Case Presentation
2.1. Chief Complaints
A 25-year-old man presented to the Emergency Department complaining of acute motor deficit of all four limbs.
2.2. History of Present Illness
The patient presented with weakness on all four limbs and an inability to deambulate. Symptoms started one year before hospitalization with mild headaches without photophobia or vomiting. Symptoms progressed rapidly with the onset of motor deficits on the right half of the body and then the involvement of the left half of the body with difficulties with speech. The patient denied fever or other constitutional symptoms.
2.3. History of Past Illness
The patient had recurrent mouth and genital ulcers going back five years before hospitalization and a history of head trauma two years before hospitalization. He was followed up for posttraumatic epilepsy and was placed on sodium valproate at a dose of 1000 mg/day.
2.4. Physical Examination
The patient’s temperature was 38.5˚C, the peripheral pulses were present and symmetrical, heart rate was 95 bpm, respiratory rate was 18 cycles per minute, blood pressure was 120/80 mmHg and oxygen saturation in room air was 99%. The neurological examination revealed a tetrapyramidal syndrome (upper motor neuron signs in all four limbs with spastic dysarthria) with cerebellar involvement (statokinetic cerebellar syndrome), a Glasgow coma scale of 15/15, without any other pathological signs. Dermatological examination revealed three oral and one genital ulceration (Figure 1). Cardiovascular and pulmonary examinations were unremarkable. Electrocardiography was unremarkable. The ophthalmologic examination did not reveal any signs of inflammation in the anterior chamber or the vitreous humor.
2.5. Laboratory Studies
The laboratory findings were as follows (Table 1): The cytogenetics study of HLA B51 was positive. Proteins C and S, and antithrombin III assays were normal. Antiphospholipid antibodies were negative.
2.6. Imaging Examination
Brain magnetic resonance imaging (MRI) revealed a large left thalamic lesion which was hyperintense on T2 and fluid-attenuated inversion recovery (FLAIR)-weighted images (Figure 2 and Figure 3).
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Figure 2. Brain MRI showing axial images demonstrating a bilateral pontine and left capsulo-thalamus lesion, hyperintense on FLAIR-weighted sequence, and little mass effect on the third ventricle.
2.7. Evolution during Hospitalization
On the fifth day of hospitalization, the patient developed a non-productive cough with polypnea. On physical examination, he was apyrexial and his respiratory rate was 50 cycles/min. A thoracic angioscan revealed a thrombosed aneurysm of the inferior lobar artery bilaterally with an obstructive intraluminal thrombus of a segmental branch of the right superior lobe (Figure 4). Transthoracic echocardiography (TTE) revealed a mobile right atrial thrombus measuring 13 × 10 mm (Figure 5).
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Figure 3. Brain MRI showing sagittal T1-weighted images with contrast enhancement of a mesencephalo-diencephalic lesion with a crown of edema and little mass effect.
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Figure 4. Chest CT with contrast revealing thrombosed aneurysm of the inferior lobar artery bilaterally with an obstructive intraluminal thrombus of a segmental branch of the right superior lobe.
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Figure 5. Transthoracic echocardiography (TTE) revealed a mobile right atrial thrombus measuring 13 × 10 mm.
3. Final Diagnosis
The diagnosis of Neuro-Behçet with cardiac and pulmonary thromboembolism was retained, according to the 2014 International Study Group Criteria for Behçet disease [7].
4. Treatment
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).
5. Outcome and Follow-Up
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.
6. Discussion
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.
7. Conclusion
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.
Acknowledgements
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.
Ethics Approval and Consent to Participate
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.
Authors’ Contributions
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.
List of Abbreviations
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