A Case of Takayasu Arteritis

B. Priyanka1,*, Vigneshvarprashanth Umapathy2,*, S. Aravindakumar3

1Duty Medical Officer, Department of Cardiology, Kauvery Heart City, Trichy, India

2Resident Internal Medicine, Kauvery Hospital, Tennur, Trichy, India

3Chief Consultant Interventional Cardiologist, Kauvery Heart City, Trichy, India

*Correspondence: [email protected] (Priyanka B); [email protected] (Vigneshvarprashanth Umapathy)

Abstract

In this report, we present a case of a 30-year-old woman admitted with headache, dyspnoea, and left upper limb pain on exertion. A diagnosis of Takayasu arteritis was made. She was started on corticosteroids and her symptoms improved.

Keywords:

Takayasu arteritis, pulseless disease, ACR criteria, large arteries, inflammation, corticosteroids

Background

Takayasu arteritis (TA), also known as pulseless disease, is a systemic inflammatory condition that leads to damage to the medium and large arteries and their branches. It occurs predominantly in young Asian women. It usually involves the aorta and its major branches, particularly the renal arteries, carotid arteries, and subclavian arteries, and leads to stenosis, occlusions, or aneurysmal degeneration of these large arteries. It is a rare disease, with a reported worldwide incidence rate of only 1 to 2 per million and the majority being females (F:M = 9:1) [1].

We present a case of Takayasu arteritis admitted at Kauvery Heart City, Trichy, India.

Case Presentation

A 30-year-old lady, known to have hypothyroidism and on thyroxine for 3 years, presented with complaints of headache and breathlessness for the past 1 week. She also had complaints of left upper limb pain on exertion for the past 3 months which was progressively worsening. She did not have any history of fever, chest pain or palpitation. CT and MRI brain taken elsewhere did not reveal any significant finding. She revealed a history of shortness of breath during her MRI scan, for which she was managed at a local hospital.

She was admitted and evaluated.

On arrival, she was conscious, oriented and dyspneic.

On examination:

Blood pressure Upper limb Lower limb
Right limb 100/70 mmHg 110/70 mmHg
Left limb 80/60 mmHg 110/60 mmHg

Heart rate: 106/min; Radial pulse (left): feeble; SpO2: 90% on room air; Respiratory rate: 26/min; GRBS: 132 mg/dl; CVS: S1S2 heard; right carotid bruit heard RS: BAE+, Bilateral crepitations heard.

She was started on diuretics and her dyspnoea started improving. Labs showed leucocytosis, elevated ESR and elevated CRP. She was started on ceftriaxone empirically.  The serial ECGs did not show significant findings. Troponin T was negative. ECHO showed moderate AR, mild MR, mild global hypokinesia of LV and mild LV dysfunction.

Takayasu arteritis was suspected. CT aortic angiography showed features suggestive of Takayasu arteritis, type II B. Doppler screening showed concentric mural thickening extending to right internal carotid artery, left external and internal carotid arteries; No evidence of flow reversal in bilateral vertebral arteries; No evidence of subclavian steal phenomenon.

Fundus examination was normal. ANA profile (immunoblot) was negative.

She was started on prednisolone 40 mg daily and methotrexate 10 mg once a week in consultation with the rheumatologist. Her symptoms resolved and was discharged in stable condition.

Important Lab Investigations

Haemoglobin 10.6 g/dl
Total WBC count 14000 cells/mm3
Platelets 367000 cells/mm3
ESR 120 mm/hr
CRP 38.3 mg/dl
ASO titre 341.84
RA factor <10.0 IU/ml
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CT Aortic angiography

: Concentric mural thickening involving the aortic arch and its branches and the proximal descending aorta and its branches, suggestive of Takayasu arteritis, type II B; Severe narrowing with occlusion of the left subclavian artery.

Discussion

With a history of left upper limb pain and findings like feeble radial pulse, carotid bruit, and elevated ESR, Takayasu arteritis was suspected and CT aortic angiography, which remains the gold standard investigation [3], confirmed the diagnosis. ACR criteria for TA were also fulfilled. Absent or feeble pulses can be found in 84-96% of the patients with TA. Vascular bruits can be heard in 80-94% of patients, often multiple, and particularly affecting the carotids, subclavian, and abdominal vessels [3]. Her dyspnoea was due to pulmonary edema which may be due to stress cardiomyopathy or Takayasu myocarditis. This also explains her dyspnoea during the MRI scan. Mild cardiomegaly with dilated main pulmonary artery was noted in CT angiography. However, ECHO did not show pulmonary artery hypertension. Her dyspnoea improved with diuretics. Her headache could be due to reduced cerebral blood flow. TA may present with neurological manifestations like dizziness, visual disturbances, ischemic stroke, and headaches in up to 30% of cases [2]. However, any evidence of cerebral vasoconstriction could not be demonstrated by CT brain and MRI brain taken outside. Manifestations in TA range may from asymptomatic disease found as a result of impalpable pulses or bruits, to catastrophic neurological impairment [3]. Her ASO titre was elevated. But the RA factor was normal. Moderate aortic regurgitation and mild mitral regurgitation were noted in the ECHO. Lili Pan et al in a retrospective study concluded that an increase in ASO titre is related to valvular involvement in TA and is closely linked to mitral insufficiency [6].

ACR criteria for Takayasu arteritis [3]

Diagnosis of Takayasu arteritis requires at least 3 of the following 6 criteria to be met.

Criteria Definition
Age at disease onset ≤40 years Development of symptoms or findings related to Takayasu arteritis at age ≤40 years
Claudication of extremities Development and worsening of fatigue and discomfort in muscles of 1 or more extremities while in use, especially the upper extremities
Decreased brachial artery pulse Decreased pulsation of 1 or both brachial arteries
Blood pressure difference >10 mm Hg Difference of >10 mm Hg in systolic blood pressure between arms
Bruit over subclavian arteries or aorta Bruit audible on auscultation over 1 or both subclavian arteries or abdominal aorta
Arteriogram abnormality Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not caused by arteriosclerosis, fibromuscular dysplasia, or similar causes; changes usually focal or segmental
taka-6-2

Angiographic classification of Takayasu arteritis

(Schematic diagram) [5]

Treatment should aim to control vascular inflammation and prevent irreversible organ damage [4]. Corticosteroids which remain the mainstay in the TA management was started in consultation with the rheumatologist. She was lot better during the follow-up.

Conclusion

Detailed history, careful examination, and relevant investigations are cardinal in the diagnosis of TA. Once diagnosed, medical management with steroids and immunosuppressive agents or revascularization procedures can be planned depending on the disease.

Take home message

  1. Takayasu arteritis is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its branches.
  2. Manifestations in TA range from asymptomatic disease, found as a result of impalpable pulses or bruits to catastrophic neurological impairment. Headache is not uncommon in TA.
  3. Angiography remains the gold standard for diagnosis.
  4. Treatment should aim to control vascular inflammation and prevent irreversible organ damage.
  5. Timely treatment with corticosteroids improves patient’s condition.

References

  1. Trinidad B, Surmachevska N, Lala V. Takayasu Arteritis. [Updated 2022 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from:https://www.ncbi.nlm.nih.gov/books/NBK459127/
  2. Barasa L, et al. Takayasu arteritis: A rare cause of chronic headache. Clinical Case Reports. 2021;9(9):1.
  3. Johnston SL. et al. Takayasu arteritis: a review. J Clin Pathol. 2002;55(481):6.
  4. Russo RA, et al. Takayasu arteritis. Front Ped. 2018;24;6:265.
  5. All about cardiovascular system and disorders, johnsonfrancis.org blog, Angiographic classification of Takayasu arteritis. Available from:https://johnsonfrancis.org/professional/angiographic-classification-of-takayasu-arteritis/
  6. Pan L, et al. Elevated antistreptolysin O titer is closely related to cardiac mitral insufficiency in untreated patients with Takayasu arteritis. BMC Cardiovascular Dis. 2020 Dec;20(1):1-8.
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Dr. B. Priyanka

Duty Medical Officer

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Dr. Vigneshvarprashanth Umapathy

Resident Internal Medicine

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Dr. S. Aravindakumar

Chief Consultant Interventional Cardiologist

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