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| CASE REPORT |
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| Year : 2022 | Volume
: 10
| Issue : 2 | Page : 124-127 |
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Takayasu arteritis in young female a case report
AV Umakanth1, Sangram Biradar2
1 Department of General Medicine, Basaveshwara Teaching and General Hospital, Attached to M R Medical College, Gulbarga, Karnataka, India
2 S B Medical Centre, Gulbarga, Karnataka, India
| Date of Submission | 03-Nov-2020 |
| Date of Decision | 18-Feb-2021 |
| Date of Acceptance | 24-Feb-2021 |
| Date of Web Publication | 02-Mar-2022 |
Correspondence Address:
Dr. Sangram Biradar
S B Medical Centre, #14, Lahoti Enclave, station Road, Gulbarga, Karnataka
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ajim.ajim_88_20
Takayasu arteritis (TA) is a systemic inflammatory large vessel vasculitis. Here, we report a rare case of 18-year-old female with complaints of upper and lower limb pain with blackish discoloration along the veins. During hospital stay, she developed breathlessness and limb pain limiting her daily activities. As her right lower limb peripheral pulses were feeble lower limb and abdominal Doppler was performed which showed thrombosis of right common femoral and superficial artery thrombosis and concentric thickening of theaorta, celiac trunk, and superior mesenteric artery, respectively. Computed tomography aortogram supported Doppler findings and two-dimensional echo showed pulmonary artery hypertension and dilated right chambers of the heart. She was started on systemic steroids and anticoagulation to which she fairly responded and she was discharged with maintenance steroids. Hence, we suggest early and prompt diagnosis of TA in young females can improve the condition and reduce the sufferings.
Keywords: Concentric wall thickening of aorta, steroids therapy, takayasu arteritis
How to cite this article:
Umakanth A V, Biradar S. Takayasu arteritis in young female a case report. APIK J Int Med 2022;10:124-7 |
| Introduction | |  |
Takayasu arteritis (TA) is a rare, systemic, inflammatory large-vessel vasculitis of unknown etiology defined as inflammatory and stenotic disease of medium and large-sized arteries characterized by a strong predilection for the aortic arch and its branches. TA most prevalent in adolescent girls and young women. It can manifest as isolated, atypical, and/or catastrophic disease and can involve any or all of the major organ systems. The disease has been reported in all parts of the world, although it appears to be more prevalent in Asians.
| Case Report | | |
An 18-year-old female patient presented with lower limb pain, painful swellings over the volar surface of the upper one-third of the bilateral forearm with associated blackish discoloration for 1 month. Pain was present over the medial part of the thigh and around both ankles, squeezing type, severe enough to restrict her daily activities, initially relieved with rest but later worsened. After a few days of the appearance of swelling, she developed mild to moderate fever, intermittent, not associated with chills, relieved with medication. With above said complaints, she was admitted to our hospital and started on supportive therapy. During hospital stay, she developed exertional breathlessness, chest pain, and diffuse pain abdomen.
On examination, there was tender firm fusiform swelling over the bilateral forearm [Figure 1], 2 cm below cubital fossa measuring 3 cm × 2 cm and there was linear blackish discoloration over the territory of greater saphenous femoral veins which was thick cord-like on palpation [Figure 2]. On peripheral pulse examination pulse over left dorsalis pedis, posterior tibial, and popliteal arteries were absent and feeble over right lower limb arteries and there was blackish discoloration over the anteromedial surface of the left ankle. There was pressure difference of 20 mmHg between the right and left arms. On cardiac examination, apex impulse was present over the left 5th intercostal space 2 cm lateral to midclavicular line loud palpable P2 with wide fixed split of S2. Diffuse tenderness was present over the abdomen.
Routine blood investigations showed thrombocytosis (8 lakh), Erythrocyte sedimentation rate (ESR) of 120 [Table 1], serology showed antinuclear antibody positivity, frontal radiograph of the chest showed cardiomegaly [Figure 3]. Electrocardiogram showed T-wave inversion in the chest leads (V1-V6), lead 3 suggestive of right ventricular strain pattern.
Lower limb Doppler was done to see the vascular patency which showed thrombosis of right common femoral and superficial femoral arteries with associated monophasic flow in both lower limb arteries and focal narrowing of great saphenous vein. Abdominal Doppler showed concentric thickening of the aorta, celiac trunk, and superior mesenteric artery. To confirm sonographic findings computed tomography aortogram was done which showed diffuse thickened wall of descending [Figure 4] abdominal aorta with diffuse narrowing of proximal superior mesenteric [Figure 5] and celiac [Figure 6] trunk bilateral narrowing of common femoral arteries [Figure 7] and double left renal artery as incidental finding while aorta and its branches were of normal caliber [Figure 8] and [Figure 9]. Two-dimensional (2D)-echo of the heart showed dilated right-sided chambers with moderate TR and pulmonary hypertension (56 mmHg). | Figure 5: Shows the narrowing of the proximal superior mesenteric artery with wall thickening noted at the origin
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 | Figure 6: Shows the narrowing of the celiac artery with wall thickening noted at the origin
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 | Figure 7: Shows very minimal opacification and narrowing of bilateral common femoral arteries
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 | Figure 9: Shows arch of the aorta which appears normal in caliber and course
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Patient was started on systemic steroids and anticoagulants for which she fairly responded and her symptoms relieved day by day. After 14 days of therapy, she got relief from lower limb pain and swellings reduced, left upper limb pulses improved, pressure difference nullified, intensity of p2 reduced, wide fixed split disappeared but lower limb pulses remained impalpable. 2D echo which was repeated showed reduction in Pulmonary artery systolic pressure (from 56 to 28 mmhg), and repeat lower limb Doppler showed recanalization of vessels.
| Discussion | | |
TA is a rare, systemic, inflammatory large-vessel vasculitis of unknown etiology that most commonly affects women of childbearing age.[1]
TA progresses through three stages starting from early systemic stage where patients may present with fatigue, malaise, fever, and other nonspecific features where it is difficult to identify disease. Second stage is a vascular inflammatory stage which may present with pain in extremities and joints, dyspnea, palpitations, headaches, rash, hemoptysis, and ulceration accompanied by symptoms of vascular insufficiency, Evidence of vessel inflammation such as tenderness along arteries, bruits, and aneurysm may point to the diagnosis of TA.[2] Third stage is burnt out stage where there is fibrosis but relapse can occur from remission and the patient can be asymptomatic.
The diagnosis of TA involves a combination of clinical, laboratory, and imaging features. Various validated criteria have been developed over the years which can help diagnose TA.
The sensitivity and specificity of the American College of Rheumatology criteria are 90.5% and 97.9%, respectively:[3]
- Age of 40 years or younger at disease onset
- Claudication of the extremities
- Decreased pulsation of one or both brachial arteries
- Difference of at least 10 mm Hg in systolic blood pressure between arms
- Bruit over one or both subclavian arteries or the abdominal aorta
- Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the upper or lower extremities that is not due to arteriosclerosis, fibromuscular dysplasia, or other causes.
Laboratory test results in individuals with TA tend to be nonspecific. The ESR may be high and frequently responds to steroids treatment.[4] Leukocyte count may be normal or slightly elevated. Normochromic anemia may be present in active disease. Cell adhesion molecules may be high in inactive cases.[5]
Angiography, the criterion standard for the diagnosis and evaluation of TA, is used to evaluate only the appearance of the lumen. TA can be divided into the following six types based on angiographic involvement.[6]
- Type I - Branches of the aortic arch
- Type IIa - Ascending aorta, aortic arch, and its branches
- Type IIb - Type IIa region plus thoracic descending aorta
- Type III - Thoracic descending aorta, abdominal aorta, renal arteries, or a combination
- Type IV - Abdominal aorta, renal arteries, or both
- Type V - Entire aorta and its branches.
Disease activity can be assessed by prospective study criteria established by Seyahi E et al. (National Institutes of Health) New onset or worsening of 2 or more of the following features indicates active disease:[7]
- Systemic features, such as fever and arthralgias (no identified cause)
- Elevated ESR
- Features of vascular ischemia or inflammation, such as claudication, diminished or absent pulse, bruit, carotidynia, or asymmetrical blood pressure in either the upper or lower limbs (or both)
- Typical angiographic features.
Others such as Birmingham Vasculitis Activity Score, Disease Extent Index for Takayasu's Arteritis (Tak), and Indian Takayasu's Arteritis Score are also being used but they are less validated.
Corticosteroids are the mainstay of therapy for active TA. However, some patients may also require cytotoxic agents. Other agents such as interleukin-6 receptor inhibitor-tocilizumab, rituximab, a chimeric immunoglobulin G1 antibody that binds to CD20, methotrexate, azathioprine, and cyclophosphamide, Anti-tumor necrosis factor agents are also being tried with variable efficacy.
| Conclusion | | |
Tak should be considered in young women with a history of lower limb pain, breathlessness, and pain in the abdomen. Timely diagnosis and treatment can alleviate patients suffering and can reduce its mortality.
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.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Tombetti E, Mason JC. Takayasu arteritis: Advanced understanding is leading to new horizons. Rheumatology 2019;58:206-19.  |
| 2. | Kothari SS. Takayasu's arteritis in children – A review. Images Paediatr Cardiol 2001;3:4. |
| 3. | Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33:1129-34. |
| 4. | Hoffman GS, Ahmed AE. Surrogate markers of disease activity in patients with Takayasu arteritis: A preliminary report from the International Network for the Study of the Systemic Vasculitides (INSSYS). Int J Cardiol 1998;66:S191-4. |
| 5. | Tripathy NK, Chandran V, Garg NK, Sinha N, Nityanand S. Soluble endothelial cell adhesion molecules and their relationship to disease activity in Takayasu's arteritis. J Rheumatol 2008;35:1842-5. |
| 6. | Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: New classification. Int J Cardiol 1996;54:S155-63. |
| 7. | Seyahi E. Takayasu arteritis: An update. Curr Opin Rheumatol 2017;29:51-6. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]
[Table 1]
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