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  IN THIS Article
 ::  Abstract
 ::  Introduction
 ::  Case report
 ::  Discussion
 ::  References

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Year : 1992  |  Volume : 38  |  Issue : 1  |  Page : 47-9,48A

Bilateral pulmonary aneurysm in Behçet's disease (a case report).

Department of Radiodiagnosis, Kasturba Medical College and Hospital, Manipal, South Kanara, Karnataka.

Correspondence Address:
B N Lakhkar
Department of Radiodiagnosis, Kasturba Medical College and Hospital, Manipal, South Kanara, Karnataka.

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Source of Support: None, Conflict of Interest: None

PMID: 0001512730

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 :: Abstract 

While investigating a chest opacity in Behcet's disease possibility of pulmonary aneurysm should be considered. Contrast enhanced CT is a safe diagnostic method which is useful in confirming or excluding the presence of an aneurysm in such cases. We report here the first case of bilateral pulmonary aneurysms in Behcet's disease.

Keywords: Adult, Aneurysm, pathology,radiography,Behcet Syndrome, pathology,radiography,Case Report, Hemoptysis, pathology,radiography,Human, Male, Pulmonary Artery, pathology,radiography,Pulmonary Embolism, pathology,radiography,Tomography, X-Ray Computed,

How to cite this article:
Lakhkar B N, Nagaraj M V, Shenoy D P, Patil U D, Ghosh M K. Bilateral pulmonary aneurysm in Behçet's disease (a case report). J Postgrad Med 1992;38:47-9,48A

How to cite this URL:
Lakhkar B N, Nagaraj M V, Shenoy D P, Patil U D, Ghosh M K. Bilateral pulmonary aneurysm in Behçet's disease (a case report). J Postgrad Med [serial online] 1992 [cited 2023 Mar 21];38:47-9,48A. Available from:

  ::   Introduction Top

Behcet's disease was first described by Hulusli Behcet[1], a Turkish dermatologist from Istanbul. It is a systemic disorder of unknown cause with vasculitis as a common pathologic process. The etiology remains uncertain but there is probably an increased incidence of HLA - B51 and HLA - DRw52[2]. It includes as major criteria recurrent oral and genital ulcers and ocular and skin lesions that may be accompanied by minor criteria such as arthropathy, neurologic manifestations and cardiovascular and gastraintestinal lesions[3]. Most cases observed occurred in Mediterranean countries, the Middle East and Japan. Pulmonary artery aneurysm is a rare but serious complication of Behcet's disease. Very few reigorts are available in the literature about this condition[4],[5]. To the best of our knowledge this is the first case of bilateral pulmonary aneurysms presenting as hilar masses in Behcet's disease.

  ::   Case report Top

A 19-year-old man presented with high grade fever, cough with expectoration and haemoptysis since 3 months. The blood loss in a bout of cough was about 20-25 milday, lasting for 5-6 days and then recurring after a gap of 2-3 days. There was a history of exertional dyspnoea of one month duration associated with vague chest pain. He gave history of repeated painful swellings of ankle, knee and elbow joints and recurrent ulcers over the scrotum, inguinal region and in the mouth. He also noticed a few non-itchy recurrent reddish skin lesions over both forearms.

He was a moderately built young man with mild pallor. There was bilateral discrete, firm, non-tender, inguinal lymphadenopathies. Multiple ulcers were seen on the scrotum, both inguinal regions and ventral aspect of penis. Small punctate ulcers were also seen over both the buccal mucosa. Rest of the examination was normal.

His Hb was 9.4gm% and total WBC count was 13,790 (N-58, E-7. L- 23.2, M-10.3, and B-0.7). ESR was 72 mm at the end of 1 hr), sputum was negative for tuberculosis and VDRL test was negative. Rest of the laboratory examination were within normal limits. An X-ray chest revealed noncalcified well defined bilateral hilar masses (See [Figure:1]), which were confirmed on tomography (See [Figure:2]). They were nonpulsatile on fluoroscopy. A CT scan at the bifurcation of right and left pulmonary artery revealed a large focal dilatation with dense intraluminal enhancement and a surrounding hypodense area suggestive of a thrombus (See [Figure:3]). A diagnosis of bilateral thrombotic pulmonary artery aneurysms was made. The patient was posted for pulmonary angiography next day but unfortunately the patient had an attack of massive haemoptysis and went into hypovolemic shock. Patient was stablised haemodynamically by blood transfusion, an emergency thoracotomy was planned. The patient continued to bleed despite supportive measures. The diagnosis of bilateral aneurysms was confirmed in the operation theatre. However, before any correitive surgery could be performed the patient bled profusely and went into hypovolemic shock from which he could not be revived. A photograph of autopsy specimen (See [Figure:4]) illustrates the aneurysmal dilatation of pulmonary arteries observed while operating.

  ::   Discussion Top

In 1947 Deterling and Clagett[6] published an extensive review of proximal pulmonary artery aneurysm. They reviewed 92,026 autopsy studies and added 17,545 of their own and concluded that only eight cases of pulmonary artery aneurysm (PAA) had been documented in a total of 109,571 autopsies. It points cut the rarity of this condition.

The most common etiological factor in preantibiotic era was infection. Tuberculosis and syphilis accounted for the majority of cases. Several bacteria and fungi have also been reported to cause pulmonary artery aneurysms. Structural cardiac abnormalities (congenital heart diseases), pulmonary hypertension and structural vascular abnormalities (congenital, cystic medionecrosis, Martour's syndrome) have high incidence ol P AA. Trauma is the next important tactor kcx the aetiology of PAA. Idiopathic causes such as Behcet's disease and Hughes-Stovin syndrome have been reported[7]. The PAA in these conditions is believed to be secondary to vasculitis.

Patients of PAA usually present with haemoptysis, dyspnea, chest pain, cyanosis and clubbing, cough and a bruit on physical examination[7]. In many cases, presenting features may be non-specific and abnormalities on chest roentgenogram may suggest possibility of pulmonary artery aneurysms. According to available literature Behcet's disease is predominant seen in men, with a peak age of onset in third decade[8]. Our patient who was a 19-year-old male, presented with cough, haemoptysis, dyspnoea and chest pain addition to features of Behcet's syndrome in the form of oral ulcerations, genital ulcerations, joint involvement and cutaneous involvement of both forearms.

Pulmonary involvement in Behcet's disease is occurring in an estimated 5% of patients[3]. It tends to ccompany exacerbation of the disease in other systems[9]. The underlying histological lesion in the lung as in other organs is a nonspecific vasculitis affecting vessels of any calibre; both arteries and veins thought to be due to immune complex deposits in the vessel wall[10]. One of the characteristics of the disease is involvement of the large vessels, mainly the veins with a frequency estimated at 24%[11]. The vena cavae are the veins most often affected[12] while very rare cases of thrombosis or aneurysms of the aorta or limb arteries have been reported[13],[14]. The pulmonary arteries affected range from the lobar and segmental branches down to the arterioles. These arteries display focal thrombosis and inflammatory lesions of the vessels with focal, transmural necrosis[5].

The histological features in the lung include perivascular infiltration, thrombosis, pulmonary infarction, aneurysm formation, haemorrhages, nodule formation, diffuse fibrosis and cavitation[9]. The radiological manifestations are accordingly varied, the commonest findings on a chest radiograph being transient alveolar infiltrates which presumably represent haemorrhage and infarction; less frequently, nodules and larger rounded opacities which tend to be central are found [9]. Our case was presented with bilateral hilar masses in plain X-rays. The margins were sharply defined and there was no evidence of any calcification. Fluoroscopy did not reveal any pulsations in the masses.

Mootoosamy et al[15] have demonstrated that CT with contrast enhancement is used for definitive diagnosis of pulmonary artery aneurysm. It is non invasive and with contrast enhancement can be used to define accurately the site, size and relationship of the aneurysm. It can also help to differentiate aneurysms from other vascular lung lesions such as pulmonary AV malformations where feeding vessels are seen. CT is also useful in diagnosing partially thrombosed aneurysms. It can demonstrate the intimate and partially concentric relationship of the soft tissue mass to the opacified arterial lumen. In our case pulmonary artery aneurysms with peripheral thrombosis could be diagnosed easily, with the help of CT Scan. This was confirmed during the surgery.

It has been stressed that PAA of any etiology, particularly when associated with haemoptysis cannot be assumed to be stable. In the absence of a controlled study, we think that the collected case histories need to be considered and they strongly favour intervention. The first rule in treatment is to correct any underlying etiologic factors that can be addressed. One must next address the pulmonary artery aneurysm itself. Treatment options vary with the location of the aneurysm with involvement of the main pulmonary trunk; Surgical intervention with aneurysmectomy is the usual tradition whereas for more peripheral lesions choice is between vascular ligation with resection of distal tissue or non surgical embolotherapy.

Stricker and Malinverni[2] have recently reported a case of multiple large pulmonary aneurysms in Behcet's disease, which was treated with 50mg of prednisone per day. After 3 months, radiologic controls (CT & DSA) showed complete resolution of the pulmonary artery aneurysms. The patient was followed upto 10 months with a tapered dose of 25mg prednisone per day. Radiographically there was no evidence of a pathologic pulmonary process. Precise Mechanism of such resolution remains unclear. However, in our opinion this line of treatment needs further evaluation in a larger series.

 :: References Top

1. Behcet H. Ueber rezidivierende. a.phthosQ. durch ein virus verursachte GeschwOre am Mund and Auge und an den Genitalien. Dermatol Wochenschr 1937; 105:1152-1157.  Back to cited text no. 1    
2.Stricker H, Malinverni R. Multiple, large aneurysms of pulmonary arteries in Behcet's disease. Clinical remission and radiologic resolution after corticosteroid therapy. Arch Int Med 1989; 149:925-927.  Back to cited text no. 2    
3.Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behcet disease (Behcet syndrome) Semin Arthritis Rheum 1979; 8:223-260.  Back to cited text no. 3    
4.Gibson RN, Morgan SH, Krausz T, Hughes GRV. Pulmonary artery aneurysms in Behcet's disease. Brit J Radiol 1985; 58:79-82.  Back to cited text no. 4    
5.Grenier P, Bletry O, Comud F, Godeau P, Nahum H. Pulmonary involvement in Beheet disease. Amer J. Roentgenol 1981; 137:565-569.  Back to cited text no. 5    
6.Deterling RA, Clagett OT. Aneurysm of the pulmonary artery, review of literature and report of case. Amer Heart J 1947; 34:471-499.  Back to cited text no. 6    
7.Bartter T, Irwin RS, Nash G. Aneurysms of the pulmonary arteries. Chest 1988; 94:1065-1075.  Back to cited text no. 7    
8.Tuzuner A, Ersoz S. A rare vascular complication of Behcet's disease: Peripheral aneurysms: Case reports. Vasc. Surg 1990; 24:610-615.  Back to cited text no. 8    
9.Cadman EC, Lundberg WB, Mitchell MS. Pulmonary manitestations In Behcet syndrome. Arch Int Med 1976; 16:944-947.  Back to cited text no. 9    
10.Gamble CN, Wiesner KB, Shapiro RE, Boyer W. The immune complex pathogenesis of glomerulonephritis and pulmonary vasculitis in Behcet's disease. Amer J Med 1979; 66:1031-1039.  Back to cited text no. 10    
11.Chajek T, Fainaru M. Behcet's disease. Report of 41 cases and a review of the literature. Medicine (Baltimore) 1975; 54:179-196.  Back to cited text no. 11    
12.Kansu E, Ozer FL, Akalin E. Behcet's syndrome with obstruction venae cavae. A report of seven cases. Quat J Med 1972; 41:151-168.  Back to cited text no. 12    
13.Hills EA. Behcet's syndrome with aortic aneurysms. Brit Med J 1967; 4:152-154.  Back to cited text no. 13    
14.Jenkins M, Maepherson AIS, Nolan B, Housley E. Peripheral aneurysms in Behcet's disease. Brit J Surg 1976; 63:199-202.  Back to cited text no. 14    
15.Mootoosamy IM, Reznek RH, Dymond DS, Rees, RSO. CT appearance of mycotic, aneurysms of the intrapulmonary arteries in the right lung. Brit J Radiol 1985; 58:85-86.   Back to cited text no. 15    

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Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
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