|Year : 2019 | Volume
| Issue : 1 | Page : 23-25
Takayasu’s Arteritis Presenting With Fever and Hemoptysis
Pritam K Chatterjee, Biswajit Majumder, Sandip Ghosh, Sudeep KN
Department of Cardiology, R. G. Kar Medical College, Kolkata, West Bengal, India
|Date of Web Publication||18-Jan-2019|
Pritam K Chatterjee
10/31 Kasundia 2nd Bylane, Shibpur, Howrah 711104 West Bengal
Source of Support: None, Conflict of Interest: None
Takayasu’s arteritis (TA) is a rare, systemic, inflammatory large-vessel vasculitis of unknown etiology resulting in a granulomatous inflammation of the aorta and its major branches. It most commonly affects the women of childbearing age. Pulmonary artery involvement is very rare. We, hereby, report a case of TA presenting with fever and hemoptysis due to pulmonary artery involvement.
Keywords: Hemoptysis, pulmonary artery, Takayasu Arteritis,
|How to cite this article:|
Chatterjee PK, Majumder B, Ghosh S, KN S. Takayasu’s Arteritis Presenting With Fever and Hemoptysis. J Assoc Chest Physicians 2019;7:23-5
| Introduction|| |
Takayasu’s arteritis (TA) is an idiopathic, inflammatory, and stenotic disease of the medium and large-sized arteries characterized by a strong predilection of the aortic arch and its branches. It is sometimes referred to as aortic arch syndrome. Apart from the involvement of the aorta, TA is the only form of arteritis to involve the pulmonary arteries. Usually, there is stenosis or an occlusion of the lobar, segmental, and subsegmental branches of the pulmonary artery, especially in the upper lobe of the lung. Dilatation of the pulmonary arteries and the main pulmonary trunk involvement are quite rare., The disease mainly affects young females. It is best known to occur in Asia and Japan. The female-to-male ratio is 6:1.
A 25-year-old female presented with low-grade fever with scanty intermittent hemoptysis for 3 months; headache, dizziness, blackouts, and generalized weakness for 2 months; and tingling and numbness in the upper limb for 1 month. On general examination, she was found to be of thin built, febrile with mild pallor. Pulsations for both the carotids and subclavian, brachial, and radial arteries were absent bilaterally. Femoral pulse was 86/min, regular, fair in volume, normal in character, and bilaterally symmetrical. Other peripheral pulses in the lower limbs were present. Bruit was present over the right carotid artery. Blood pressure was not recordable in the upper limbs. In the lower limb, it was 160/90 mmHg. Systemic examination revealed no other abnormalities.
On investigation, complete hemogram showed elevated erythrocyte sedimentation rate (ESR) and normocytic normochromic anemia. C-reactive protein level was elevated. A sputum stain for acid-fast bacillus was negative. GeneXpert was performed, and the result was negative. Tests on antinuclear antibody and anti-ds-deoxyribonucleic acid (DNA) antibodies were negative. An echocardiogram showed mild pulmonary arterial hypertension. A Doppler study of the neck vessels showed an occlusion of the innominate, left common carotid and the left subclavian arteries (LSCA). Computed tomography (CT) aortogram was performed, which showed diffuse wall thickening and an enhancement of the ascending and descending thoracic aorta and the aortic arch [[Figure 1]A and B]. The innominate artery and the bilateral common carotid artery (CCA) showed a thickened wall with occlusion [[Figure 2]A]. The right CCA showed gross thickening with an enhancement of the wall up to the bulb. The left CCA showed long-segment stenosis from origin. There was total occlusion of the right subclavian arteries and LSCA and from origin, and the pulmonary trunk was dilated with an occlusion of the right pulmonary artery [[Figure 2]B]. The abdominal aorta was normal. Chest X-ray and high resolution computed tomography (HRCT) thorax were normal [Figure 3].
|Figure 1 (A) CT aortogram showing diffuse wall thickening and an enhancement of the ascending and descending thoracic aorta and the aortic arch. (B) CT aortogram showing diffuse wall thickening and an enhancement of the arch of aorta|
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|Figure 2 (A) CT aortogram showing the thickening of the walls of the innominate, bilateral common carotid artery (CCA) and the left subclavian artery with occlusion. (B) CT angiography showing the dilated pulmonary trunk with an occlusion of the right pulmonary artery|
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|Figure 3 Chest X-ray and high resolution computed tomography (HRCT) thorax|
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A diagnosis of TA with pulmonary arterial involvement was made, and she was put on a high dose of corticosteroids along with symptomatic therapy. The patient is currently doing well and is on regular follow-up.
| Discussion|| |
TA is an idiopathic, inflammatory, and stenotic disease of the medium- and large-sized arteries characterized by a strong predilection of the aortic arch and its branches. It is sometimes referred to as aortic arch syndrome. It mainly affects young adults, especially females.
TA can involve the pulmonary artery (PA) in 10–40% of the patients. In Indian studies, PA involvement has been reported in 15–27% of the patients. Usually, a stenosis/occlusion of the lobar, segmental, or subsegmental branches was involved, especially of the right side. The dilatation of the pulmonary trunk and an occlusion of the main PA are extremely rare. In pulmonary TA, extensive collateral circulation among the pulmonary, bronchial, inferior phrenic, intercostal, and coronary arteries has been demonstrated. Patients can present with exertional breathlessness and hemoptysis.,, In India, because tuberculosis is very much prevalent, any patient presenting with fever and hemoptysis pulmonary tuberculosis needs exclusion. In our case, the absence of pulse in the upper limbs was the clue to the diagnosis of TA. This case was unique, because it involved the main pulmonary trunk, which was dilated, and an occlusion of the right pulmonary artery from its origin. Very few cases of main pulmonary artery involvement are reported.
| Conclusion|| |
Though a histopathologic involvement of the pulmonary artery is not very uncommon in TA, clinical manifestation in the form of hemoptysis is rare. Moreover, in a country such as India, in any patient with fever and hemoptysis, pulmonary tuberculosis is a much more common etiology. In our case, the absence of pulses in the upper limbs was the clue to the diagnosis of TA. In any patient with fever and hemoptysis, a meticulous general physical examination, especially of the peripheral pulses, is important to avoid unnecessary investigations and missing a relatively rare disease. A simple routine examination of the arterial pulsation of all extremities often gives us clue for the diagnosis of vascular diseases.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]