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Year : 2023  |  Volume : 19  |  Issue : 1  |  Page : 29-31

A case of Takayasu arteritis presenting with sensorineural hearing loss as first manifestation

Department of Internal Medicine, Sahyadri Superspeciality Hospitals, Nagar Road, Yerwada, Pune, Maharashtra, India

Date of Submission25-Jan-2023
Date of Decision21-Feb-2023
Date of Acceptance22-Feb-2023
Date of Web Publication28-Mar-2023

Correspondence Address:
Priya Baluni
Department of Internal Medicine, Sahyadri Superspeciality Hospitals, Nagar Road, Yerwada, Pune 411006, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AMJM.AMJM_1_23

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We report the case of a 26-year-old male patient diagnosed with Takayasu arteritis after 4 years of first manifestation. He developed sensorineural hearing loss in left ear, followed 2 years later by acute onset paraparesis and hypertension, and followed another 2 years later by short duration of upper backache, chest pain, palpitations, and breathlessness. On examination, he had hypertension without any significant blood pressure (BP) difference within arms with all peripheral pulses palpable. Diagnosis confirmed with computerized tomography aortography showing multifocal wall thickenings in entire aorta. In our case, the first presentation was otological followed 2 years later by hypertension. Our report points toward fact that hypertension, though the most common, may not be the first manifestation and characteristic examination finding of BP difference, and pulselessness may not be seen in all cases. High risk of suspicion and early use of specific investigation in cases of multisystem involvement in young hypertension are crucial for timely diagnosis.

Keywords: Hypertension, sensorineural hearing loss, Takayasu arteritis

How to cite this article:
Baluni P, Duggal D. A case of Takayasu arteritis presenting with sensorineural hearing loss as first manifestation. Amrita J Med 2023;19:29-31

How to cite this URL:
Baluni P, Duggal D. A case of Takayasu arteritis presenting with sensorineural hearing loss as first manifestation. Amrita J Med [serial online] 2023 [cited 2023 Jun 4];19:29-31. Available from: https://ajmonline.org.in/text.asp?2023/19/1/29/372701

  Introduction Top

Takayasu arteritis is a rare chronic large vessel vasculitis with incompletely understood etiopathogenesis. Aorta with its major branches is predominantly affected. Disease progresses in three phases; first systemic phase is marked by constitutional symptoms followed by vasculitis phase, in which there are features of vascular involvement like tenderness over vessels. Final phase is fibrotic or burnt-out phase. However, this temporal progression is seen only in minority of patients. There is remission of active phase of the disease spontaneously in about 3 months or it may progress insidiously for months to years into a chronic phase. There can be coexistence of inflammatory and fibrotic changes.[1] It is important to diagnose the disease during the first two phases to prevent progression into the third phase when typical examination findings appear, and our case report highlights the need for a high index of suspicion for timely diagnosis.

  Case Report Top

We report the case of a 26-year-old male patient referred to our tertiary care center, with complaints of chest pain, breathlessness, and palpitations for 1 day on a background history of mild to moderate intensity of upper back and both shoulder pain since 4 weeks. Patient had sensorineural hearing loss in left ear diagnosed as Meniere’s disease 4 years back. The patient was diagnosed with transverse myelitis and hypertension 2 years back when he had sudden onset of weakness of both lower limbs associated with loss of pain and temperature sensations below the umbilicus. magnetic resonance imaging spine revealed demyelinating lesion traversing D2–D10 level. The patient was treated with glucocorticoids and mycophenolate mofetil for 6 months. Antihypertensives were continued (telmisartan 40 mg and amlodipine 5 mg once daily). Patient examination showed raised blood pressure (BP) 190/100 mmHg in right upper limb without significant BP difference in both arms. All peripheral pulses were palpable with high pulse rate (130/min), residual weakness (power 4/5) in left lower limb, and complete recovery of weakness in right lower limb, with bilateral plantar extensor.

On investigations high resolution computed tomography chest showed bilateral mild pleural effusion and minimal pericardial effusion, raised D-Dimer (22,436 ng/ml), ultrasonography abdomen showed infrarenal aortic dilation measuring 8 cm in length and 3.7 cm in diameter. Two-dimensional echocardiography showed mild concentric left ventricle hypertrophy 60% ejection fraction. Computerized tomography angiography (CTA) [Figure 1] showed multifocal wall thickenings in entire aorta, origins of three aortic arch branches and bilateral common iliac arteries. Focal wall calcifications were seen in descending aorta. Infrarenal abdominal aorta up to its bifurcation (80 mm length) appeared dilated and measured 34.5 mm in diameter suggesting aneurysm. Inflammatory markers showed raised erythrocyte sedimentation rate (90 mm) and C reactive protein (81.81 mg/L). Hemogram showed raised total leucocyte count (15,600) and liver function test and kidney function test were unremarkable. Cardiac markers were normal. Covid reverse transcriptase polymerase chain reaction was negative, and dengue serology, NS1 antigen, venereal disease research laboratory, hemoglobins antigen, hepatitis c virus antibodies, and Human Immunodeficiency Virus Ag/Ab combo were nonreactive.
Figure 1: CTA 3D image showing abdominal aortic aneurysm

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Autoimmune workup was negative for anti nuclear antibodies, perinuclear anti neutophilic cytoplasmic antibodies, cytoplasmic anti neutophilic cytoplasmic antibodies, lupus anticoagulant, beta 2-glycoprotein I (IgM, IgG), Mi-2, SS-A, Scl-70, Jo-1, dsDNA, and AMA-m2 (mitochondrial). C3 and C4 complement levels were normal.

18-flouro deoxy glucose positron emission tomography (PET) CT showed mild wall thickening involving aorta, proximal left common iliac, proximal left femoral arteries with multiple nodular wall calcifications, and without significant flouro deoxy glucose tracer uptake.

On the basis of above findings, the patient was diagnosed with Takayasu arteritis and treated with antihypertensives and steroids-pulse therapy for 3 days followed by prednisolone 60 mg once daily for 1 month followed by taper of 5 mg/week for 10 weeks with maintenance on 5 mg/day. Symptoms resolved within 4 weeks.

  Discussion Top

In recent years, the prognosis of Takayasu arteritis is getting improved with decreasing mortality rates. Vascular intervention rates are still broadly different in clinical series.[2] As a group of inflammatory disorders of blood vessels, systemic vasculitis may show ear manifestations as well. Hearing impairment may be followed by tinnitus and vertigo. Hearing loss can be sensorineural or conductive with severity ranging from mild impairment to complete deafness.[3] One recent case report from Japan described a 37-year-old male patient who developed bilateral sensorineural hearing loss with tinnitus 14 months before admission. CT images demonstrated diffuse aortic wall thickening and he was diagnosed with Takayasu arteritis.[4] Another case report by Bayat et al.[5] described a 28-year-old female patient having Takayasu arteritis complicated with sensorineural hearing loss and patient received cochlear implantation for same. In a recently published case series by Mendis et al.[6] in autoimmune vestibulopathy has considered Takayasu arteritis as a cause for autoimmune inner ear disease but the exact prevalence is unknown. In India, male patients with Takayasu arteritis exhibit higher frequency of hypertension with abdominal aorta involvement and female patients show more involvement of aortic arch and its branches.[7] Sharma and Jain[8] showed that hypertension and its related symptom of headache were the most common presentation. Goel et al.[9] showed that most common presenting features were hypertension, headache, malaise, and fever. A total of 62.5% of the cases were having pulseless disease. Another study, which compared clinical manifestations of Takayasu arteritis in Indians and Japanese patients, showed major proportion of Indian patients presented as hypertension. Angiography suggested that Indian patients had mainly involvement of abdominal aorta and its branches as compared to aortic arch and its branches involvement in Japanese patients.[10]

Assessment of disease activity relies on clinician impression supported by composite clinical disease activity indices.[11] In our patient, the disease activity assessment showed raised erythrocyte sedimentation rate and C reactive protein with clinical symptoms and signs of active disease; however, on PET-CT the lesions did not show active uptake of flouro deoxy glucose. Measuring disease activity accurately with the help of acute phase response markers though advocated lacks both sensitivity and specificity. One study showed that 23% of Takayasu arteritis patients had normal laboratory parameters and still active disease. Erythrocyte sedimentation rate was raised in only 72% of patients considered to have active disease and was still high in 44% of patients considered to have inactive disease.[12]

Role of imaging investigation to measure disease extent, activity, and therapeutic effects is also reviewed extensively. Unfortunately, no single imaging investigation is ideal. Intra-arterial catheter-based dye angiography though gold standard, is invasive and now replaced by magnetic resonance angiography, CTA, and PET scanning.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Vaideeswar P, Deshpande JR. Pathology of Takayasu arteritis: A brief review. Ann Pediatr Cardiol 2013;6:52-8.  Back to cited text no. 1
Alibaz-Oner F, Direskeneli H. Update on Takayasu’s arteritis. Presse Med 2015;44(6 Pt 2):e259-65.  Back to cited text no. 2
Rahne T, Plontke S, Keyßer G. Vasculitis and the ear: A literature review. Curr Opin Rheumatol 2020;32:47-52.  Back to cited text no. 3
Nomura S, Ichikawa T, Tsukada K, Kishida M, Kondo Y, Shimoshima Y, et al. Takayasu arteritis with hearing impairment: A case report and its clinical background. Shinshu Med J 2021;69:261-6.  Back to cited text no. 4
Bayat A, Daneshi A, Karimi M, Saki S, Saki N. Cochlear implantation in an adult woman with Takayasu’s arteritis: A case report. Aud Vestib Res 2021;30:309-13.  Back to cited text no. 5
Mendis S, Longley N, Morley S, Korres G, Kaski D. Autoimmune vestibulopathy—A case series. Brain Sci 2022;12:306.  Back to cited text no. 6
Sharma BK, Jain S. A possible role of sex in determining distribution of lesions in Takayasu arteritis. Int J Cardiol 1998;66:S81-4.  Back to cited text no. 7
Sharma BK, Sagar S, Singh AP, Suri S. Takayasu arteritis in India. Heart Vessels 1992;7:37-43.  Back to cited text no. 8
Goel R, Kumar TS, Danda D, Joseph G, Jeyaseelan V, Surin AK, et al. Childhood-onset Takayasu arteritis—Experience from a tertiary care center in South India. J Rheumatol 2014;41:1183-9.  Back to cited text no. 9
Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestations of Takayasu arteritis in India and Japan—New classification of angiographic findings. Angiology 1997;48:369-79.  Back to cited text no. 10
Misra DP, Wakhlu A, Agarwal V, Danda D. Recent advances in the management of Takayasu arteritis. Int J Rheum Dis 2019;22:60-8.  Back to cited text no. 11
Direskeneli H, Aydın SZ, Merkel PA. Assessment of disease activity and progression in Takayasu’s arteritis. Clin Exp Rheumatol 2011;29:S86-91.  Back to cited text no. 12


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