Intracardiac thrombus in Behcet’s disease: A rare case in the United States
Article Outline
Abstract
Behcet’s disease is a rare systemic disorder which is associated with an underlying vasculitis. Most cases of BD occur in Asia and North American cases are rare. Patients often present with aphthous ulcers and have a number of other clinical findings including genital lesions, skin lesions (folliculitis, erythema nodosum, and acne), and uveitis. Hemoptysis and cough in BD may be a sign of pulmonary involvement with include pulmonary artery aneurysms (PAA). Intracardiac thrombus is another rare occurrence in BD but should be suspected if there is a right heart mass detected on imaging. We present a case of suspected BD who presented with hemoptysis and was diagnosed with PAA and intracardiac thrombus. His aphthous ulcers were not diagnoses until subsequent hospitalization for hemoptysis. Clinicians should be aware of unusual presentations of BD because of the associated high mortality.
Keywords: Behcet’s disease, Hemoptysis, Intracardiac thrombus, Pulmonary aneurysms
1. Introduction
Behçet’s disease (BD) is a systemic inflammatory disorder caused by underlying vasculitis that is often overlooked in the United States.1 Mean age of onset is between 25 and 30 years of age. Previously thought to be more prevalent in males, the disease equally affects men and women.2 Most cases of BD have been diagnosed in the Mediterranean, Middle East, and Japan; geographically known as the Silk Road.1 The incidence of this disorder in North America is 1 in 1,000,000 people. Both genetic and environmental factors may predispose some individuals to BD.2 Hemoptysis is the main presenting symptom and aphthous oral ulcers are the most common presenting physical exam finding.3
2. Case report
A previously healthy 27-year-old male with noncontributory past medical history presented with a six month history of dry cough and intermittent hemoptysis. Hemoptysis ranged from speckled sputum to a cup of bright red blood once a day. For several weeks prior to hospitalization he experienced severe exertional dyspnea that limited his mobility. The patient also complained of left sided chest pain that radiated to his shoulder while coughing. He had 40 pounds of unintentional weight loss over the preceding two months. Social history included 10 pack years of tobacco abuse, however patient had quit three years previously. On Physical examination the patient appeared weak and thin, in mild respiratory distress. Vital signs were as follows: temperature 98.2 °F; blood pressure 128/70 mmHg; heart rate 117 bpm; respiratory rate 20 bpm; oxygen saturation 100% on room air. HEENT: no JVD, clear oral pharynx, no oral lesions. Chest: normal heart sounds without any murmurs and lung fields were clear to auscultation. GU: no penile ulcers, otherwise unremarkable. GI: no abnormalities. Neuro: alert, oriented, no focal findings. Skin: the patient had folliculitis of the face.
Laboratory findings included the following: white blood cell count, 12.59 × 103 cells/μL with normal differential; hemoglobin, 12.4 gm/dL; hematocrit, 38.5%; platelets, 384 × 103 cells/μL; Erythrocyte sedimentation rate, 57 mm/HR. Basic metabolic profile, coagulation profile, liver function panel and rapid HIV were within normal limits. Initial chest x-ray found prominent right hilar round opacities. CT with contrast showed multiple bilateral central pulmonary artery aneurysms with superimposed pulmonary emboli (Fig. 1). The right upper lobe and lower lobe pulmonary artery aneurysms measured 19 mm and 23 mm, respectively. Lower lobe pulmonary artery aneurysms demonstrated no patent vessels. Echocardiography showed severe right ventricle enlargement and a 3.9 cm × 1.5 cm friable mass with a broad attachment to the right ventricular outflow tract and distal right ventricle, with multiple mobile components. There was mild to moderate trabeculation of the right ventricle. The mass appeared vascularized on color doppler and was most consistent with cardiac tumor. MRA with contrast confirmed the mass was vascularized, with intense gadolinium enhancement.
Fig. 1
CT chest with contrast demonstrating multiple bilateral central pulmonary artery aneurysms with superimposed pulmonary embolisms. These findings are consistent with BD.
3. Discussion
In 2003, the BD Research Committee of Japan revised the 1987 criteria for diagnosis.2 The major symptoms are recurrent oral aphthous ulcers, skin, ocular, and genital lesions. Minor symptoms include arthritis, epididymitis, CNS, vascular, and GI lesions. The lack of pathognomic symptoms or definitive laboratory tests make it a difficult and sometimes overlooked diagnosis.2 BD can exist as complete, incomplete or BD suspected; depending on what clinical findings appear during the disease course. Pathergy skin test is highly specific to BD with lower sensitivities in Western countries. Insertion of a needle into the subcutaneous skin causes an exaggerated response from neutrophil hyper-reactivity.2 Skin responsiveness is more pronounced during stages of active disease. This patient did not have positive pathergy skin test findings.
On a second admission for hemoptysis the patient had developed painful oral ulcers thus a diagnosis of suspected BD made based on the aphthous oral ulcers and the folliculitis of the skin.2 Large vessel involvement is observed in 15–35% of patients with BD. Sensitivity of vascular involvement in BD is low but specificity of arterial occlusion and/or aneurysm (98%) is higher than those of eye lesions (93%), a positive pathergy test (90%) and most skin lesions (75%; without erythema nodosum).4, 5 Large vessel involvement might be observed earlier than oral ulcerations like in our case.
The vasculitis of BD is immune complex mediated and may affect both arterial and venous vessels of all sizes. One fourth of BD patients eventually develop large vessel pathology, the foremost cause of morbidity and mortality.3, 6 Arterial and cardiac complications, including aneurysms, arteritis, and thrombosis, are rare but life threatening.3 Deep venous thrombosis is the most common large vascular lesion in BD, affecting one fourth of patients.7 The aorta, followed by the pulmonary artery, is the most commonly affected artery.3 The venous lesions are mainly occlusive, while arterial lesions are aneurysmal or occlusive. Cardiac involvement occurs in 1–7% of patients with reported complications including right ventricle thrombosis, endomyocardial fibrosis, pulmonary artery thrombosis, pericarditis, myocarditis, coronary thrombosis, and valvular disease.7, 8
The most common pulmonary manifestation and one of the leading causes of death is pulmonary artery aneurysm (PAA). The resulting hemoptysis, up to 500 ml, may be due to bronchial erosion secondary to aneurismal rupture. PAAs are primarily found in the right lower lobe, but also in the right and left main pulmonary arteries.6 In BD patients with PAAs, the occurrence of large extra pulmonary vessel involvement is significantly higher versus those without PAAs.6, 9
The PAAs may be seen on chest CT and angiography as saccular or fusiform dilations. The aneurysms develop secondary to inflammation of the vasa vasorum of the tunica media, leading to luminal dilation from destruction of the elastic fibers.9 This signals a dismal prognosis as 30% of these patients will die within 2 years. Despite therapeutic modalities, approximately 50% of patients will die within 2 years once hemoptysis starts.6, 9 Anticoagulant treatment for presumed PE can result in massive hemorrhage and death. There is speculation regarding the etiology of pulmonary artery thrombosis, they are thought to be in situ thrombosis, not embolic.9 Although DVTs are common, pulmonary emboli are rare from the DVTs because thrombi of the lower extremities are strongly affixed in BD.9
An intracardiac thrombus should always be considered in the differential of a right heart mass as early diagnosis and treatment are essential in BD.8 This is a rare, but serious complication, with less than 50 reported cases.10 Right ventricular thrombi are usually associated with pulmonary vasculitis. Biopsies are helpful in determining the pathological features of BD.7, 10 A right ventricular biopsy was not performed on our patient as it involved excess risk and management would not be affected by the results.
For treatment, anticoagulant and thrombolysis are considered for intracardiac thrombus.8 However, because recurrences of intracardiac thrombus are reported in cases that were treated by anticoagulant only, systemic glucocorticoid and/or immunosuppressant are highly recommended before using anticoagulant.8 Medical treatment with immunosuppressive agents is preferred over surgery because a recurrent aneurysm or fistula at the anastomotic site is a common complication after surgical resection.3 Due to our patient’s continued hemoptysis, anticoagulation was contraindicated.
In conclusion, this patient who presented with hemoptysis and cough was ultimately diagnosed with Behçet’s Disease based on clinical and radiographic findings, after he later developed oral aphthous ulcers. Although an uncommon diagnosis particularly in the United States, it may also be under diagnosed. This type of vasculitis has the potential to affect several organ systems and should be part of the differential diagnosis when a patient is found to have aneurysms, arteritis, and thrombosis. Although anticoagulation was not used in this case, it is a common treatment approach along with corticosteroids.
Conflict of interest
All authors declare that they have no conflicts of interest.
References
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PII: S1755-0017(11)00038-8
doi:10.1016/j.rmedc.2011.07.001
© 2011 Elsevier Ltd. All rights reserved.
