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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 16  |  Issue : 1  |  Page : 105-108

A fatal case of right subclavian artery aneurysm due to takayasu's arteritis presenting as brachial plexopathy


1 Department of Medicine, Command Hospital Air Force, Bengaluru, Karnataka, India
2 Department of Neurology, Command Hospital Air Force, Bengaluru, Karnataka, India

Date of Submission01-Dec-2020
Date of Acceptance25-Dec-2020
Date of Web Publication23-Mar-2021

Correspondence Address:
Dr. Prashant Kumar Dixit
Department of Medicine, Command Hospital Airforce, Agram Post, Bengaluru - 560 007, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/injr.injr_324_20

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  Abstract 


A 36-year-old lady presented with insidious onset, gradually progressive neuropathic pain, sensory loss, and weakness of the right upper limb. Her brachial, ulnar, and radial pulses were not palpable on the right side and feeble on the left. She had an irregular, ill-localized 8 cm × 6 cm pulsatile swelling in the right supraclavicular region with thrill on palpation and bruit on auscultation. Motor system examination revealed grade 4 power with wasting of thenar muscles. Nerve conduction studies were suggestive of pan-brachial plexus involvement. Computed tomography angiogram images revealed thickening of the aortic arch, irregular narrowing of the descending aorta, and aneurysm of the right subclavian artery. She was diagnosed to be suffering from right brachial plexopathy due to compression by aneurysm of the right subclavian artery secondary to Takayasu's arteritis. The case is rare as no such presentation has been reported earlier.

Keywords: Brachial plexus, subclavian aneurysm, sudden death, Takayasu's arteritis


How to cite this article:
Dixit PK, Gupta S, Kumar M H, Goel J. A fatal case of right subclavian artery aneurysm due to takayasu's arteritis presenting as brachial plexopathy. Indian J Rheumatol 2021;16:105-8

How to cite this URL:
Dixit PK, Gupta S, Kumar M H, Goel J. A fatal case of right subclavian artery aneurysm due to takayasu's arteritis presenting as brachial plexopathy. Indian J Rheumatol [serial online] 2021 [cited 2021 Apr 16];16:105-8. Available from: https://www.indianjrheumatol.com/text.asp?2021/16/1/105/311310




  Introduction Top


Brachial plexus is a plexus formed by the fusion of spinal nerves from the cervical (C5–C8) to upper thoracic (T1) nerve roots. It innervates the muscles and skin of the upper extremity.[1] The etiology of brachial plexopathy is divided into traumatic and nontraumatic causes. Nontraumatic causes are tumor (primary or secondary), cervical rib, aneurysms, radiation, neuralgic amyotrophy, hereditary brachial plexopathy, infections, and diabetes related.[1],[2] In a case series of 203 patients, the reported common causes of brachial plexopathies were brachial neuritis (40%), birth-related injuries (22%), traumatic (20%), postsurgery (8%), tumors (3.5%), radiation (5%), and cervical rib (1.5%) with localization at the upper trunk (27%), lower trunk (11%), upper/middle trunk (11%), and all trunks (25%).[3] Takayasu's arteritis (TA) is a type of vasculitis that affects large vessels and commonly involves the aorta with its branches, subclavian arteries, and carotids.[4] It causes chronic granulomatous inflammation likely autoimmune in origin; however, the exact etiology is unknown. Clinical features associated with TA are fever, weight loss, anorexia, fatigue, malaise, claudication of upper limbs, absence of peripheral pulses, bruit on auscultation, hypertension, heart failure due to hypertension/aortic regurgitation and neurological, pulmonary, and gastrointestinal system involvement.[5],[6] Subclavian artery aneurysm is not very common and has an incidence ranging from 0.13% to 1%.[7] Here, we report an unusual case of brachial plexopathy who presented with clinical features of the right brachial plexus involvement in the background of fever and constitutional symptoms.


  Case Report Top


A 36-year-old female with no known prior illness presented with complaints of pain in the right upper limb of 3 months' duration. The pain was insidious in onset, was moderate to severe in intensity, started as a pain in the right scapular region with radiation to the right hand, and increased in severity for 10 days before presentation with partial relief after taking analgesics. Two months after the onset of pain, she noticed progressive weakness of the right hand with numbness in the medial four fingers sparing the thumb. There was a history of intermittent fever, anorexia, and unintentional weight loss of 2 months' duration. There was no history of weakness of any other limb, a band-like sensation in the neck, sensory level on the trunk, or bowel/bladder incontinence. She denied a history of rashes, photosensitivity, oral ulcers, joint pains, trauma to the right shoulder, and radiation exposure.

Her examination revealed a pulse of 88/min regular; her brachial, ulnar, and radial pulses were not palpable on the right side and feeble on the left with other peripheral pulses palpable. Blood pressure on the right upper limb was unrecordable, left upper limb 160/82 mmHg, right lower limb 156/80 mmHg, and left lower limb 158/82 mmHg. General examination revealed pallor, irregular ill-localized 8 cm × 6 cm pulsatile swelling in the right supraclavicular region with thrill on palpation and bruit on auscultation. Motor system examination revealed atrophy of the right thenar and interossei muscles, grade 4 power in the right deltoid, supraspinatus, biceps, triceps, supinator, and wrist flexion (flexor carpi ulnaris, flexor carpi radialis), and grade 4 + power in the right wrist extensors with weak thenar, hypothenar, and interossei muscles. Power at all muscle groups in the other limbs was normal. She had normal deep tendon reflexes and flexor plantar. Sensory examination revealed diminished sensation (small and large fiber) in the right medial side of the arm and forearm more than the lateral side of the arm and forearm.

Laboratory investigations revealed elevated erythrocyte sedimentation rate (ESR) of 120 mm/1/h and C-reactive peptide (CRP) of 48 mg/L. Her complete blood count and liver function and renal function tests were within normal limits. Her nerve conduction study (NCS) revealed unrecordable combined motor action potential (CMAP) in the right median, ulnar, and radial nerve with decreased CMAPs of the deltoid, biceps, and supraspinatus as well as unrecordable sensory nerve action potential in the right radial, medial antebrachial cutaneous, lateral antebrachial cutaneous, median, and ulnar nerves. NCS was suggestive of pan-brachial plexus involvement at the level of roots (C8T1 > C5C6C7). Electromyogram was not done. Right upper limb Doppler showed biphasic low peak systolic velocity in the subclavian, axillary, brachial, radial, and ulnar arteries. Computed tomography (CT) angiogram images of the neck, chest, and abdomen revealed aneurysm of the right subclavian artery measuring 7.7 cm × 6.6 cm, circumferential wall thickening of major branches of aorta (brachiocephalic, subclavian, and common carotid) with calcified plaques causing focal/segmental narrowing/stenosis, there were features of thickening of the aortic arch, irregular narrowing of descending aorta, and hiatal segment of aorta showing aneurysmal dilatation with dissection/ partial thrombosis of false lumen/intramural hematoma [Figure 1]a, [Figure 1]b and [Figure 2]a, [Figure 2]b. Positron emission tomography (PET)-CT showed evidence of metabolically active aneurysmal dilatation involving the brachiocephalic trunk and right proximal subclavian artery [Figure 3]a, [Figure 3]b. It also showed dilatation of proximal abdominal aorta with metabolically active vascular wall thickening with evidence of dissection with severe luminal narrowing in the left subclavian artery, left common carotid artery, and celiac artery at their origin. Magnetic resonance imaging with the contrast of the cervical spine with whole-spine screening did not reveal any clinically significant lesion of the spine or spinal nerves. Two-dimensional echocardiography revealed mild concentric left ventricle hypertrophy with normal ejection fraction.
Figure 1: Computed tomography angiogram of the chest in the coronal section showing (a) aneurysm of the right subclavian artery (blue arrow), (b) thickening and enhancement of the wall of the arch of aorta (orange arrow)

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Figure 2: Computed tomography angiogram of the chest in the sagittal section showing (a) maximum intensity projection image of the right subclavian artery showing aneurysm (red arrow), (b) maximum intensity projection image showing irregular narrowing of descending, thoracic and abdominal aorta, calcification within vessel wall (green arrow), multiple aneurysms of the suprarenal abdominal aorta (orange arrow)

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Figure 3: (a) Whole-body positron emission tomography showing metabolically active right subclavian artery aneurysm (red arrow) in the coronal section, (b) metabolically active right subclavian artery aneurysm in the sagittal section (red arrow)

Click here to view


The patient was diagnosed to have brachial plexopathy based on the clinical evidence of involvement of lower motor neuron type motor weakness with sensory loss in the right upper limb supported by NCS finding of right-sided pan-brachial plexus involvement and MRI spine ruling out spinal cord involvement. She was classified as TA based on fulfilling 5 out of 6 criteria of the American College of Rheumatology criteria (1990) with disease activity of 11 calculated by using the Indian Takayasu Clinical Activity Score (2010). The reason for brachial plexopathy was compression by aneurysm of the right subclavian artery.

She was managed with immunosuppressants with intravenous (iv) methylprednisolone 500 mg for 5 days, followed by oral prednisolone as maintenance therapy. In view of severe disease, she was given injection tocilizumab 8 mg/kg iv single dose. She received supportive/symptomatic care for the neuropathic pain, physiotherapy, and other supportive care. After a comprehensive discussion with the surgical team, a decision to not perform any procedure directed toward an aneurysm was taken in view of active severe disease. She was discharged with advice to follow-up after 30 days for the next dose of tocilizumab. However, after 14 days while at home, she suddenly collapsed and succumbed to her illness.


  Discussion Top


Here, we report a rare presentation of an uncommon disease. A patient with brachial plexopathy typically presents with motor and sensory deficits involving the upper extremity based on the site of brachial plexus involvement. Aneurysm of the subclavian artery due to any cause is a rare event, with a reported incidence of 0.13%–1% of all the aneurysms.[7] Subclavian aneurysm as a cause of brachial plexopathy is very rare and mostly reported in various case reports in the literature.[7],[8] Aneurysms in TA mostly occur in the aorta, and various studies have reported an incidence of subclavian artery aneurysm from nil to 6.3%.[5],[9],[10] In TA, the aorta with its branches, subclavian artery, carotid, and mesenteric arteries, is most commonly affected.[11] We did not find any case of brachial plexopathy because of a subclavian artery aneurysm due to TA. Only one case report of brachial plexopathy due to axillary artery aneurysm secondary to TA has been reported, and one report of peripheral neuropathy due to TA is available.[11],[12] Neurological involvement in TA is mostly of the central nervous system due to ischemia caused by disease affecting carotid or vertebral arteries.[13]

While managing this case, we were “between the devil and the deep blue sea” situation. The patient had the active disease as indicated by elevated inflammatory markers (CRP of 48 mg/L, ESR 120 of mm/h fall) with features of active disease on PET scan and extensive involvement of aorta, brachiocephalic trunk, and aneurysm of the right subclavian artery. The mainstay of treatment of TA is medical management with steroids and steroid-sparing agents (methotrexate, leflunomide, mycophenolate mofetil, azathioprine, and cyclophosphamide). Biological agents are also used in TA, especially in refractory disease. Various biological agents that have been tried for refractory TA are anti-tumor necrosis factor (TNF) agents (infliximab, etanercept), tocilizumab (interleukin-6 inhibitor), rituximab, and ustekinumab (interleukin-12 inhibitor). Maximum studies have been done with anti-TNF agents and tocilizumab, and these two are recommended for the refractory TA with equal efficacy and safety profile.[14],[15] There are limited studies with the use of rituximab and ustekinumab, but the initial results are favorable.[16],[17] Results with the use of tocilizumab are more encouraging as compared to steroid/steroid-sparing agents, with fewer side effects and longer disease-free period.[14],[15] The definitive treatment to reduce brachial plexus compression and decompression of subclavian artery aneurysm is surgical/endovascular intervention. The surgical reconstruction and endovascular interventions are indicated in uncontrolled hypertension due to renal artery stenosis, severe symptomatic coronary artery or cerebrovascular disease, severe aortic coarctation or regurgitation, stenotic or occlusive lesions resulting in critical limb ischemia, ischemia related to coeliac or mesenteric artery, and aneurysms at risk of rupture.[18],[19] The use of surgical reconstruction or endovascular intervention depends on the artery involved, expertise/experience of the surgeon, and facilities available in the hospital. Endovascular intervention in the form of percutaneous transluminal balloon angioplasty (PTBA) with or without stenting is preferred for short segment stenotic lesions of renal, subclavian, and sometimes carotid arteries, while endovascular aneurysm repair is another option for aneurysms.[14],[18],[19] Surgical reconstruction is indicated for long-segment stenotic lesions and aortic disease (aneurysm, dissection, and coarctation). The major surgical procedures are bypass graft (aortocervical, cervicosubclavian, aortocoronary, aortoaortic, or aortorenal), aortic valve repair/replacement, and reconstruction of aortic aneurysm/dissection.[20] Both the interventions are associated with high complication rates and are difficult to perform because of thickened, fibrosed vessel wall, and periarterial fibrosis.[5],[14] The common complications associated with both the procedures are restenosis, bleeding, infection, and stroke. In this case, after extensive discussion with the surgical team, a combined decision to defer the surgical intervention till control of active inflammation was made. The pros for the surgery/endovascular intervention in this patient were severe symptoms due to compression of right brachial plexus by subclavian artery aneurysm; while cons were increased chances of perioperative rupture of an aneurysm of the subclavian artery and poor healing secondary to active inflammation of the vessel wall. As a general rule, surgery/endovascular intervention is recommended at the time of quiescent disease, when the inflammatory markers are not elevated to reduce complications.[11],[21]

Our patient succumbed to her illness while at home, for which, no cause could be identified, as an autopsy could not be performed. Literature search on the sudden death in TA patients revealed very limited results with case reports on death due to myocardial infarction (MI) and heart failure.[22],[23] Common causes of death in a TA patient are due to cardiovascular causes (MI, heart failure) and stroke (cerebral infarction and hemorrhage).[24] The cause of death in this case could probably be due to rupture of an aneurysm, MI, or aortic dissection. The patient had an aortic dissection of hiatal and proximal segment of abdominal which can be seen in CT angiography and PET-CT, thereby making it one of the possible causes of sudden death. Now, the question which came to our mind was “could we have prevented her death?” It is difficult to answer, and based upon the available evidence, we deferred her surgery till the control of disease with medical management.

To the best of our knowledge, this is the first reported case of brachial plexopathy due to the subclavian artery aneurysm caused by TA. This case highlights the importance of the rare occurrence of brachial plexopathy due to an uncommon disease (TA).

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Tarng DC, Huang TP, Lin KP. Brachial plexus compression due to subclavian pseudoaneurysm from cannulation of jugular vein hemodialysis catheter. Am J Kidney Dis 1998;31:694-7.  Back to cited text no. 8
    
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Mekinian A, Comarmond C, Resche-Rigon M, Mirault T, Kahn JE, Lambert M, et al. Efficacy of biological-targeted treatments in Takayasu arteritis: Multicenter, retrospective study of 49 patients. Circulation 2015;132:1693-700.  Back to cited text no. 15
    
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Jedidi M, Chkirbene Y, Abdessayed N, Masmoudi T, Mahjoub M, Mlayeh S, et al. Sudden death due to unusual complication of Takayasu arteritis: An autopsy case. Am J Forensic Med Pathol 2017;38:91-3.  Back to cited text no. 23
    
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Park SJ, Kim HJ, Park H, Hann HJ, Kim KH, Han S, et al. Incidence, prevalence, mortality and causes of death in Takayasu arteritis in Korea – A nationwide, population-based study. Int J Cardiol 2017;235:100-4.  Back to cited text no. 24
    


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