Indian Journal of Radiology and Imaging Indian Journal of Radiology and Imaging

THORACIC AND CARDIOVASCULAR IMAGING
Year
: 2002  |  Volume : 12  |  Issue : 2  |  Page : 215--217

Pulmonary manifestations of Behcet's disease


M Gulati, NBS Mani, P Singh, S Suri 
 Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
M Gulati
Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh 160012
India




How to cite this article:
Gulati M, Mani N, Singh P, Suri S. Pulmonary manifestations of Behcet's disease.Indian J Radiol Imaging 2002;12:215-217


How to cite this URL:
Gulati M, Mani N, Singh P, Suri S. Pulmonary manifestations of Behcet's disease. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Jul 2 ];12:215-217
Available from: http://www.ijri.org/text.asp?2002/12/2/215/28448


Full Text

 Introduction



Behcet's disease is a rare multisystemic disorder of unknown etiology characterized by recurrent attacks. Although the triple symptom complex of oral and genital ulcerations with uveitis was reported earlier, clinical manifestations additional to this triad were described later including involvement of the skin, joints, large vessels, lung, brain, gastrointestinal tract and genitourinary tract. It is now recognized as a multisystem disease with vasculitis as the main pathological finding[1]. Haemoptysis of varying degrees (upto 500 ml) is the most common and predominant symptom of a pulmonary aneurysm.

 Case Report



A 28-year-old male presented with a history of dry cough, dyspnoea and intermittent haemoptysis of three months duration. He gave past history of a sudden onset of left sided hemiplegia and altered sensorium for which an MRI was performed and revealed multiple hypodense areas in the brain stem suggestive of infarcts. He had no history of oral or genital ulcers, arthralgia, photosensitivity, skin rash, eye or renal disease.

On examination, the patient was tachypnoeic. He had pallor, bilateral pedal edema and acneform skin lesions over the back and chest. He was normotensive and the peripheral pulses were equally palpable in all the four limbs.

An arterial blood gas analysis showed mild hypoxemia and respiratory alkalosis. Renal and liver function tests were normal. Tests for rheumatoid factor, antinuclear antibodies (ANA), anti-neutrophilic cytoplasmic antibodies (ANCA), Anti-glomerular basement membrane antibodies (Anti-GBM), HIV and HBsAg were negative. Urine examination showed a trace of albumin. Pathergy test (Pustular reaction of the skin to an intradermal needle prick) was negative.

A chest radiograph showed dilated pulmonary arteries on both sides [Figure 1]a. Computed tomographic(CT) scans of the chest revealed aneurysmal dilatation of both main pulmonary arteries and their major branches with thickening of the dilated vessel walls along with thrombi within the lumen of these aneurysms [Figure 1]b. Axial MR images (MRI) with gadolinium enhancement revealed similar findings [Figure 2]a. These findings were better appreciated on MR angiography of the thorax [Figure 3]a.

A diagnosis of multiple pulmonary artery aneurysms with vessel wall involvement secondary to vasculitis was made and the patient was started on oral prednisolone (1mg/kg/day). He refused cyclophosphamide therapy because of possible gonadal dysfunction. He reported significant symptomatic improvement at the end of three months of the therapy. His effort tolerance improved and the haemoptysis subsided. A repeat chest radiograph done 6 months later showed disappearance of the hilar prominence [Figure 4]a. A repeat MR angio of the thorax demonstrated significant reduction in the size of the aneurysms and disappearance of the intraluminal thrombi [Figure 3]b. The size of the pulmonary vessels had returned to normal.

 Discussion



In view of the pulmonary aneurysms, CNS involvement, acneform skin lesions, hypodense lesions in the brain stem and response to systemic steroids, a diagnosis of Behcet's disease was made in this patient. Behcet's disease is a rare multisystemic disorder of unknown etiology characterized by recurrent attacks. Although the triple symptom complex of oral and genital ulcerations with uveitis was reported earlier, clinical manifestations additional to this triad were described later including involvement of the skin, joints, large vessels, lung, brain, gastrointestinal tract and genitourinary tract. It is now recognized as a multisystem disease with vasculitis as the main pathological finding[1]. Haemoptysis of varying degrees (upto 500 ml) is the most common and predominant symptom of a pulmonary aneurysm. Rupture of an aneurysm with erosion into a bronchus and development of in situ thrombosis from active vasculitis have been suggested as an explanation for the haemoptysis[1]. Recurrent oral ulcerations although a common finding, may not be present in all the patients. A Positive Pathergy test favours the diagnosis of Behcet's disease strongly but a negative test does not rule out the diagnosis as it can be negative in 70% of the patients[2].

Imaging patients presenting with an aneurysmal form of the disease has also been a challenge. Chest x-ray films usually reflect the aneurysms as hilar prominences, polylobular or round opacities (that sometimes resemble enlarged lymph nodes[4]. Digital subtraction angiography has also been used in the diagnosis but it may be inadequate if the aneurysm or vessels are completely thrombosed. Numan et al in their study of 15 patients with Behcet's disease having pulmonary aneurysms showed that MR imaging is better than DSA in presumptive diagnosis of occluded aneurysms present in two of their patients[4].

Imaging techniques such as aortography, venography and pulmonary angiography are no longer used as they carry a risk of complications[1]. Severe deterioration following angiography has been reported in half of the patients undergoing the procedure[3]. The necessity of intravenous contrast agent is a significant disadvantage. Helical CT is an excellent non invasive technique having an advantage of decreased motion artefact and use of a small quantity of contrast material. MRI and MRA offer the obvious advantage of multiplanar imaging capability without the need for contrast injection[5].

Previous studies on MRA of aneurysmal disease have suggested that the best results may be obtained by a combination of T1 weighted SE sequences and MRA techniques. T1 weighted SE images are helpful to locate the aneurysm, show its extent and to plan optimal location and orientation of breath hold MRA images[5].

References

1Erkan F, Gul A, Tasali E. Pulmonary manifestations of Behcet's disease. Thorax 2001; 56:572-578.
2Eugene V Ball. Behcet's disease. Cecil: Textbook of Medicine, 21st edition; 1540-1541.
3Erkan F, Cavdar T. Pulmonary vasculitis in Behcet's disease. Am Rev Respir Dis 1992; 146:232-239.
4Numan F, Islak C, Berkmen T, Tuzun H, Cokyuksci O. Behcet disease: pulmonary arterial involvement in 15 cases. Radiology 1994; 192 (2): 465-468.
5Berkmen T. MR Angiography of aneurysms in Behcet Disease: A report of four cases. JCAT 1998; 22 (2): 202-206.