INTRODUCTION
Takayasu’s arteritis (TA) is an infrequently occurring form of large vessel vasculitis linked to inflammation in the walls of the aorta and its primary branches, resulting in a reduction in blood flow and subsequent affectation (1). This leads to a pulseless disease with claudication, compromised peripheral pulses, and frequent ischemia (2).
TA is rare, with some studies reporting an incidence of 1 patient per 1 million inhabitants and tends to be more common in women, with the onset of symptoms typically occurring between the ages of 15 and 25 (3).
Neurological involvement can occur in half of the patients, and strokes are a common complication of Takayasu’s arteritis (4). In this paper, we describe the case of a male patient suffering from Takayasu’s arteritis, who had a severe ischemic stroke as a manifestation.
CASE REPORT
In this paper, we present a case of a male in his mid-fifties with a history of generalized myalgias and arthralgias, accompanied by headaches and cervical pain that had worsened over the previous month. Due to the abrupt development of right-sided hemiplegia and motor aphasia, he was admitted to our hospital. No other systemic symptoms accompanied the patient’s presentation.
During the physical examination, his vital signs revealed a normal heart and respiratory rate, with no need for oxygen support. However, it was unusual that the blood pressure in his right arm was recorded as 72/55 mmHg. The patient was non-verbal but responsive when it came to simple commands, and he exhibited right hemiplegia. Brain computed tomography (CT) showed the absence of hemorrhaging and mass effect. The patient was treated within a 4-hour timeframe from the onset of the stroke with alteplase, and there were no complications, but there was also no improvement in his neurological status.
Brain and neck angiography revealed an infarction in the territory of the left middle cerebral artery, along with a substantial occlusion of the left common carotid artery and the left subclavian artery, with extensive collaterals of the vertebral arteries and basilar artery. No signs of aortic dissection were observed. (Figure 1).
Brain magnetic resonance imaging (MRI) showed a left hemispheric stroke in the left middle cerebral artery (MCA), involving the temporal region, without any hemorrhagic transformation. (Figure 2).
This pattern was highly suggestive of Takayasu’s arteritis. In a focused physical examination, the patient exhibited a wide pulse deficit and a difference in blood pressure of >10 mmHg between the two arms.
Laboratory tests revealed a normal hemogram with no electrolyte abnormalities. However, the erythrocyte sedimentation rate (ESR) was elevated at 40 mm/hour (normal range: 0–15 mm/hr). The rheumatological profile showed negative results for anti-nuclear antibodies (ANA), including extractable nuclear antigens (ENA) and double-stranded DNA (dsDNA). Additionally, vasculitis studies indicated negative results for anti-neutrophil cytoplasmic antibodies (ANCA).
With the findings of a pulseless disease, elevated erythrocyte sedimentation rate (ESR), and arterial compromise observed on a CT angiogram, the patient was diagnosed with Takayasu’s arteritis. Treatment was initiated with high-dose steroids and cyclophosphamide (5), due to the unavailability of biologic therapies targeting TNF-alpha and IL-6 at the hospital. At the patient’s follow-up at the Department of Rheumatology, the therapeutic plan was adjusted, with the introduction of tocilizumab into therapy. Two months later, the patient demonstrated partial recovery of movement but continued to experience aphasia. Currently, he is undergoing an intense rehabilitation program.
DISCUSSION
Takayasu’s arteritis presents as a pulseless disease and it was first described in 1908 by Mikito Takayasu as “a case of peculiar changes in the central retinal vessels.” In this condition, the pulse is altered due to inflammation of the wall of the aorta and its major branches (6). The etiology of Takayasu’s arteritis is still idiopathic (7).
The clinical manifestations of Takayasu’s arteritis (TA) can vary widely, ranging from asymptomatic to severe neurological complications. There are also differences in presentation between younger and older patients (those over 40 years of age), with diagnosis often being delayed in the latter group (1). Therefore, it is important to investigate the signs of generalized inflammation syndrome in patients with TA who present with symptoms such as fever, night sweats, malaise, anorexia, weight loss, and diffuse myalgias, as this condition can often be misdiagnosed as an infection (7). In the case of our patient, frequent cervical pain and headaches were associated symptoms.
It is important to emphasize the triphasic pattern of the disease, as patients often present during the pre-pulse inflammatory phase with non-specific systemic symptoms (fever, arthralgia, and weight loss). Due to this, they are initially diagnosed with a prolonged viral syndrome (8). However, in the case of our patient, systemic symptoms were not considered until neurological complications developed.
Approximately 50% of patients suffering from Takayasu’s arteritis can experience neurological symptoms, with visual symptoms being the most prevalent. The neurological presentation is so forgotten that there are Neurorheumatology books in which there is no description of it. (9) Strokes can affect 10% of the patients. The presentation of embolic disease in Takayasu’s arteritis has been associated with stenotic or occlusive lesions in the aorta and its branches. Additionally, it is linked to subsequent hypertension or eventual cerebral hypoperfusion (4).
Our patient experienced a manifestation of the disease with a massive ischemic stroke. According to the criteria outlined by the American College of Rheumatology in 1990, he met five of them, which led to the suspicion of the diagnosis of Takayasu’s arteritis. This was not the sole factor leading to the diagnosis; considering the radiological characteristics, other potential causes of medium vessel vasculitis were taken into account. In the differential diagnosis of cranial artery involvement, distinguishing between Takayasu’s arteritis and giant cell arteritis (GCA), it’s crucial to note that GCA primarily does not impact intracranial (i.e., cerebral) arteries (10).
Standard treatment with high-dose steroids and cyclophosphamide as additional immunomodulators was initiated (11). However, corticosteroids need to be withdrawn as soon as possible to avoid side effects, and it is preferable to consider new biological therapies, especially tocilizumab, an IL-6 inhibitor, in order to improve the course of the disease (12).
CONCLUSSION
Nowadays, the physical exam has been relegated to a secondary role, but there are certain diseases where an exhaustive physical examination could easily lead to an early clinical diagnosis. In this report, we emphasize the severity of Takayasu’s arteritis in a young male patient, a condition that could have a different course if diagnosed during the pre-pulse inflammatory phase.
Acknowledgments: The authors report no acknowledgments.
Funding: For this work authors did not receive any funding.
Informed Consent Statement: The authors have obtained the written informed consent from from the patient mentioned in the article. The corresponding author is in possession of this document.
Conflict of interest statement: The authors declare no conflict of interest.
REFERENCES / LITERATURA
<jrn>1. Mason JC. Takayasu arteritis - advances in diagnosis and management. Nat Rev Rheumatol. 2010 July;6(7):406–15.PubMedhttps://doi.org/10.1038/nrrheum.2010.82</jrn>
<jrn>2. Quinn KA, Gribbons KB, Carette S, Cuthbertson D, Khalidi NA, Koening CL, et al. Patterns of clinical presentation in Takayasu’s arteritis. Semin Arthritis Rheum. 2020 August;50(4):576–81.PubMedhttps://doi.org/10.1016/j.semarthrit.2020.04.012</jrn>
<jrn>3. Onen F, Akkoc N. Epidemiology of Takayasu arteritis. Presse Med. 2017 July;46(7-8 Pt 2):e197–203.PubMedhttps://doi.org/10.1016/j.lpm.2017.05.034</jrn>
<jrn>4. Hwang J, Kim SJ, Bang OY, Chung CS, Lee KH, Kim DK, et al. Ischemic stroke in Takayasu’s Arteritis: Lesion patterns and possible Mechanisms. J Clin Neurol. 2012;8(2):109.PubMedhttps://doi.org/10.3988/jcn.2012.8.2.109</jrn>
<jrn>5. Saadoun D, Bura-Riviere A, Comarmond C, Lambert M, Redheuil A, Mirault T, et al. French recommendations for the management of Takayasu’s arteritis. Orphanet J Rare Dis. 2021 July 21;16 Suppl 3:311.PubMedhttps://doi.org/10.1186/s13023-021-01922-1</jrn>
<jrn>6. Sugiyama K, Ijiri S, Tagawa S, Shimizu K. Takayasu disease on the centenary of its discovery. Jpn J Ophthalmol. 2009 March 1;53(2):81–91.PubMedhttps://doi.org/10.1007/s10384-009-0650-2</jrn>
<jrn>7. Alnabwani D, Patel P, Kata P, Patel V, Okere A, Cheriyath P. The Epidemiology and Clinical Manifestations of Takayasu Arteritis: A Descriptive Study of Case Reports. Cureus. 2021 September 15;13(9):e17998.PubMedhttps://doi.org/10.7759/cureus.17998</jrn>
<jrn>8. Quinn KA, Gribbons KB, Carette S, Cuthbertson D, Khalidi NA, Koening CL, et al. Patterns of clinical presentation in Takayasu’s arteritis. Semin Arthritis Rheum. 2020 August;50(4):576–81.PubMedhttps://doi.org/10.1016/j.semarthrit.2020.04.012</jrn>
<edb>9. Cho TA, Bhattacharyya S, Helfgott S, editors. Neurorheumatology. Cham: Springer International Publishing; 2019.</edb>
<jrn>10. Larivière D, Sacre K, Klein I, Hyafil F, Choudat L, Chauveheid MP, et al. Extra- and Intracranial Cerebral Vasculitis in Giant Cell Arteritis. Medicine (Baltimore). 2014;93(28):e265.PubMedhttps://doi.org/10.1097/MD.0000000000000265</jrn>
<jrn>11. Dua AB, Kalot MA, Husainat NM, Byram K, Springer JM, James KE, et al. Takayasu Arteritis: a Systematic Review and Meta‐Analysis of Test Accuracy and Benefits and Harms of Common Treatments. ACR Open Rheumatol. 2021;3(2):80–90.PubMedhttps://doi.org/10.1002/acr2.11186</jrn>
<jrn>12. Mekinian A, Saadoun D, Vicaut E, Thietart S, Lioger B, Jego P, et al. Tocilizumab in treatment-naïve patients with Takayasu arteritis: TOCITAKA French prospective multicenter open-labeled trial. Arthritis Res Ther. 2020;22(1):218.PubMedhttps://doi.org/10.1186/s13075-020-02311-y</jrn>
Figure 1. Neck and brain CT angiogram. Stenosis involving the origin of the left common carotid artery and left subclavian artery, with extensive collaterals of the vertebral arteries and basilar artery
Figure 2. Brain magnetic resonance image (MRI). Evolving infarct involving the area of the middle cerebral artery (MCA)