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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 3  |  Page : 316-318

Humeral artery aneurysm revealing a rare association between tuberculosis and behçet's disease


1 Department of Internal Medicine, Maamouri Hospital, Nabeul, Tunisia
2 Department of Plastic, Hand Surgery and Burns, Maamouri Hospital, Nabeul, Tunisia
3 Department of Radiology, Maamouri Hospital, Nabeul; Radiology Department, Medical University of Tunis Manar, Tunisia

Date of Submission08-Apr-2020
Date of Decision20-May-2020
Date of Acceptance20-May-2020
Date of Web Publication28-Aug-2020

Correspondence Address:
Rabie Ayari
Department of Plastic, Hand Surgery and Burns, Maamouri Hospital, Nabeul
Tunisia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmy.ijmy_71_20

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  Abstract 


The association of pulmonary tuberculosis and Behçet's disease revealed by an aneurysm of the humeral artery is exceptional with a complicated management. We report a case in which the two conditions occurred concomitantly with the vascular complication, apart from any use of immunosuppressive therapy, something that has never been reported in the literature. We report an extremely rare case of a spontaneous rupture of an aneurysm of the humeral artery of a 29-year-old woman with no history. The patient underwent axillo-humeral bypass. Investigations concluded the diagnosis of Behçet's disease associated with pulmonary and lymph node tuberculosis. Antituberculous chemotherapy followed by corticosteroids, immunosuppressants, and colchicine has been administrated. Based on this observation, we insist on the necessity of searching the symptoms of Behçet's disease in the presence of arterial involvement when having a young patient. Therapeutic management must include medical treatment to control inflammation and limit the risk of recurrence. Endovascular or surgical treatment is necessary if the arterial involvement is threatening. The association with tuberculosis complicates management and requires close monitoring.

Keywords: Aneurysm, Behçet's disease, humeral artery, imaging, tuberculosi


How to cite this article:
Amri R, Ayari R, Triki R, Chaabane I, Bouzaidi K, Sbai MA. Humeral artery aneurysm revealing a rare association between tuberculosis and behçet's disease. Int J Mycobacteriol 2020;9:316-8

How to cite this URL:
Amri R, Ayari R, Triki R, Chaabane I, Bouzaidi K, Sbai MA. Humeral artery aneurysm revealing a rare association between tuberculosis and behçet's disease. Int J Mycobacteriol [serial online] 2020 [cited 2020 Sep 21];9:316-8. Available from: http://www.ijmyco.org/text.asp?2020/9/3/316/293548




  Introduction Top


Behçet's disease is an inflammatory, systemic vasculitis originally described by the Turkish dermatologist Hulusi Behçet in 1937. It is typically characterized by the combination of recurrent oral and genital aphtosis with ocular involvement. Vascular involvement in Behçet's disease occurs in 5%–40% of cases depending on the series[1] and is associated with increased mortality.[2] Venous thrombosis is frequently observed, while arterial damage such as aneurysms, pseudoaneurysms, strictures, and occlusions is less reported.[2] Arterial involvement in Behçet's disease mainly affects the aorta and pulmonary arteries. The humeral artery is extremely rarely affected.

The association of Behcet's disease and tuberculosis is rare and often correlated with the use of immunosuppressants.

To our knowledge, there is no reported case in the literature of spontaneous rupture of an aneurysm of the humeral artery of a patient having Behçet's disease and tuberculosis. Thus, we report the first case revealing this association.


  Case Report Top


We report the case of a 29-year-old woman with no history who presented with a right humeral mass of 50 mm × 70 mm evolving for 2 months and that has spontaneously ruptured. An axillohumeral bypass using the basilic vein was made. The intraoperative findings were consistent with an aneurysm of the humeral artery. The axillary artery had an inflammatory aspect with a very thickened wall suggesting vasculitis, and the humeral artery downstream was narrowed.

Histological analysis of the resected lesions showed a nonspecific panvascularitis.

Investigations carried out postoperatively revealed recurrent bipolar aphtosis associated with a 2-year history of inflammatory arthralgia and a 2-month history of claudication of the left upper limb. Physical examination showed a mouth ulcer on the inside of the lower lip [Figure 1], scars of genital ulcers, and pseudofolliculitis. Peripheral pulses were present, symmetrical but weak in the left upper limb. Pathergy test was positive.
Figure 1: Mouth ulcer on the inside of the lower lip

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Arterial Doppler objectified the patency of the left axillary, subclavian, humeral, radial, and ulnar arteries which were thin with damped and demodulated spectra. A thoracoabdominopelvic computed tomography angiography was performed in search of other vascular lesions [Figure 2] and [Figure 3]. It showed a saccular aneurysm of the left subclavian artery, bronchiolar micronodules, and thoracic and subdiaphragmatic lymphadenopathies, the largest of which contained central necrosis and some of which were calcified. The diagnosis of Behçet's disease with mucocutaneous and arterial involvement associated with tuberculosis was made. Antituberculous chemotherapy followed by corticosteroids and immunosuppressants (cyclophosphamide and azathioprine), as well as colchicine, has led to an uneventful recovery without recurrence.
Figure 2: Thoracic computed tomography angiography showing a saccular aneurysm of the left subclavian artery (cross section)

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Figure 3: Computed tomography angiography of the left upper limb showing: fusiform aneurysm of the subclavian artery, occlusions of the left humeral artery of over 13 cm and occlusion of the lower two-third of the left ulnar artery (reconstruction image)

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  Discussion Top


Vascular involvement during Behçet's disease affects preferentially young males with a sex ratio male: female: 5:1.[3] It is more frequent in the Middle East and Mediterranean countries. Our patient is a 36-year-old woman.

Most often, vascular involvement in Behçet's disease affects the venous system, usually in the form of thrombosis. Arterial involvement affects around 10% of patients and makes the severity of the disease.[4] The main arterial lesions are aneurysms, occlusions, and more rarely, arterial stenosis or diffuse aortitis.[5]

Anatomopathological examination often shows active lesions made of an inflammatory infiltrate preferentially affecting the media, the adventitia, and the surrounding of the vasa vasorum. It is associated with scar lesions with fibrous thickening in the media, the adventitia, and the intima, all leading to the distension of the walls and the constitution of aneurysms or pseudoaneurysms.

Aneurysms are by far one of the most serious and feared complications of Behçet's disease, especially the pulmonary location. All arteries can become aneurysmal; the main localizations are aortic, femoral, pulmonary, iliac, popliteal, and subclavian. The other rarer arterial localizations are digestive, coronary, cerebral, and upper limbs.[6],[7] In the series of Saadoun et al.,[7] the arterial lesions of the lower limbs were more frequent than those of the upper limbs with, respectively, 51 cases versus 5. Nevertheless, we should mention that the arterial localizations are willingly multiple in approximately 30% of the cases.[6]

The rupture of the aneurysms of the humeral artery is dominated by traumatic causes (direct contusion, arteriography, arterial catheterization, blood gas, pulmonary biopsy, and arterial bypass). In our case, the spontaneous rupture of the aneurysm of the humeral artery would probably be due to its chronicity and its voluminous size.

Ultrasound coupled with color and pulsed Doppler is the key examination and the first to request in front of a pulsatile mass, and it allows the diagnosis to be made by showing an increase in the caliber of the artery.

Behçet's disease should be suspected when having a young patient with an arterial aneurysm of the upper limb. Clinical signs in favor should be sought by vigorous interrogation and examination to avoid delayed diagnosis leading to serious complications.

The choice between endovascular and surgical treatment of aneurysmal lesions is not well codified, but it should ideally be made at a distance from the acute phase. However, many of these lesions are diagnosed at the stage of rupture or prerupture requiring emergency surgery which is the case of our patient.

Control of inflammation is essential; therefore, a high dose of corticosteroid therapy is recommended for serious arterial damage in combination with immunosuppressants.[8]

The association of Behçet's disease with aneurysmal involvement in pulmonary tuberculosis is very rarely reported in the literature[9] and concerns the pulmonary arteries in most cases. The involvement of the humeral artery in the case of our patient is the first ever to be described in the literature in our knowledge.

The rare cases of association of Behçet's disease with other pathologies and notably infectious such as pulmonary tuberculosis have been particularly observed when treatments such as tumor necrosis factor-a blockers or other immunosuppressants were newly introduced, which was not the case in our observation which makes it special.

Apart from immunosuppressive therapy, Efthimiou et al.[10] explain this association by the disruption of the immune system induced by the disease itself by genetic predisposition and by the ethnic factor which seems to be important in our case since Tunisia is a tuberculosis endemic country. All of thse highlight the value of an anti-bacillary prophylactic treatment in the case of immune diseases.


  Conclusion Top


Arterial involvement during Behçet's disease is one of the main causes of mortality and morbidity. Aneurysms are the most common form, mainly affecting the aorta, the pulmonary, and femoral arteries. The arteries of the upper limbs are rarely affected, but they can inaugurate the disease and even be life-threatening in case of spontaneous or traumatic rupture, hence the importance of suspecting Behçet's disease when facing any arterial aneurysm of the upper limb of a young patient. Pulmonary or extrapulmonary tuberculosis can be associated with Behçet's disease even outside the use of an immunosuppressive therapy. Here comes the utility to carry out screening tests or even to administer prophylactic treatment based on Isoniazid in endemic countries before any other medication.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Düzgün N, Ateş A, Aydintuǧ OT, Demir O, Olmez U. Characteristics of vascular involvement in Behçet's disease. Scand J Rheumatol 2006;35:65-8.  Back to cited text no. 1
    
2.
Koç Y, Güllü I, Akpek G, Akpolat T, Kansu E, Kiraz S, et al. Vascular involvement in Behçet's disease. J Rheumatol 1992;19:402-10.  Back to cited text no. 2
    
3.
Ko GY, Byun JY, Choi BG, Cho SH. The vascular manifestations of Behçet's disease: Angiographic and CT findings. BJR 2000;73:1270-4.  Back to cited text no. 3
    
4.
Park JH, Chung JW, Joh JH, Song SY, Shin SJ, Chung KS, et al. Aortic and arterial aneurysms in Behçet disease: Management with stent-grafts – Initial experience. Radiology 2001;220:745-50.  Back to cited text no. 4
    
5.
Huong DL, Wechsler B, Papo T, Piette JC, Bletry O, Vitoux JM, et al. Arterial lesions in Behçet's disease. A study in 25 patients. J Rheumatol 1995;22:2103-13.  Back to cited text no. 5
    
6.
Desbois AC, Wechsler B, Cacoub P, Saadoun D. Inflammatory aortic involvement associated with Behçet's disease. Rev Méd Interne 2016;37:230-8.   Back to cited text no. 6
    
7.
Saadoun D, Asli B, Wechsler B, Houman H, Geri G, Desseaux K, et al. Long-term outcome of arterial lesions in Behçet disease: A series of 101 patients. Medicine (Baltimore) 2012;91:18-24.  Back to cited text no. 7
    
8.
Desbois AC, Wechsler B, Cacoub P, Saadoun D. Aortic inflammatory lesions in Behçet's disease. Rev Med Interne 2016;37:230-8.  Back to cited text no. 8
    
9.
Haouel M, Kooli C, Azzabi S, Cherif E, Kaoueche Z, Hassine LB, et al. Behcet's disease complicated by pulmonary artery aneurysm: About 4 cases. Société Fransaised Ethumatologie; Ma.76;2008.   Back to cited text no. 9
    
10.
Efthimiou J, Hay PE, Spiro SG, Lane DJ. Pulmonary tuberculosis in Behçet's syndrome. Br J Dis Chest 1988;82:300OME.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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Abstract
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