Case Report

Perforation of the Nasal Septum in a Patient with Takayasu’s Arteritis: A Case Report and Literature Review

Boseung Jung1https://orcid.org/0000-0001-7669-6575, Jung Soo Kim1https://orcid.org/0000-0002-0899-3794, Sung Jae Heo1,*https://orcid.org/0000-0002-1388-8075
Author Information & Copyright
1Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyungpook National University, Daegu, Korea
*Corresponding author: Sung Jae Heo, Department of Otorhinolaryngology-Head and Neck Surgery, Kyungpook National University Chilgok Hospital, Daegu 41404, Korea, Tel: +82-53-200-2792, Fax: +82-53-200-2027, E-mail: blueskyhsj@hanmail.net

© Copyright 2023 xxxxxxxxxxxxxxx. This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received: Aug 10, 2023; Revised: Sep 04, 2023; Accepted: Nov 06, 2023

Published Online: Dec 31, 2023

ABSTRACT

Takayasu’s arteritis is a chronic inflammatory arteriopathy affecting the aorta and its major branches, leading to stenosis or occlusion of the major arteries and causing ischemic symptoms. Nasal septal perforation is known to have various causes and to occur in patients with diverse rheumatic diseases such as Wegener’s granulomatosis, relapsing polychondritis, and systemic lupus erythematosus. However, septal perforation in Takayasu's arteritis is extremely rare. A 48-year-old woman without any other underlying disease presented nasal septal perforation (18×14 mm) associated with Takayasu’s arteritis. Septal perforation was treated conservatively, and Takayasu's arteritis was treated with immunosuppressive drugs, so septal perforation did not worsen and the patient did not experience any discomfort. Although septal perforation is extremely rare in Takayasu's arteritis, if other causes of perforation are excluded, Takayasu's arteritis also can be considered as a cause for septal perforation.

Keywords: Nasal septal perforation; Nasal septum; Takayasu arteritis

Introduction

Takayasu’s arteritis is a type of chronic inflammatory arteriopathy that occurs in the aorta and its major branches, resulting from various causes.1) In the early stages of the disease, nonspecific symptoms manifest, whereas in the later stages, several complications can occur. Complications caused by arterial stenosis, occlusion, and aneurysm formation may be accompanied by the absence of peripheral pulses, claudication, auscultatory murmurs, hypertension, Raynaud’s phenomenon, stroke, aortic regurgitation, congestive heart failure, pericarditis, and myocardial infarction.2)

Nasal septal perforation can be caused by trauma, nasal septal surgery, inflammatory diseases, tumors, systemic disorders, toxic substances, etc.3) It has also been reported to occur in patients with various rheumatic diseases. Nasal septal perforation is one of the major clinical manifestations of Takayasu’s arteritis along with Wegener’s granulomatosis and relapsing polychondritis.4) In patients with systemic lupus erythematosus, nasal septal perforation has been reported as a complication resulting from mucosal involvement, with an incidence rate of approximately 5%.5) Septal perforation has also been infrequently reported to occur in patients with juvenile idiopathic arthritis, sarcoidosis, rheumatoid arthritis, and adult-onset Still’s disease.1)

Septal perforation associated with Takayaus’s arteritis is extremely rare and has not been reported in Korea. In this context, we aimed to report a case of coexisting nasal septal perforation and Takayasu’s arteritis in a 48-year-old woman along with a literature review.

Case Report

A 48-year-old previously healthy woman presented with intermittent headaches for the past 7 years. She recently experienced a significant increase in headache severity and thus sought medical evaluation. Brain magnetic resonance imaging (MRI) performed in the neurology department did not show any acute lesions. However, magnetic resonance angiography (MRA) of the brain revealed bilateral internal carotid artery occlusion and collateral blood flow in the bilateral posterior communicating arteries. Based on these findings, the patient was admitted to the neurology department of our hospital. Physical examination showed absence of pulsation in the bilateral brachial arteries, right carotid artery, bilateral radial arteries, and ulnar arteries, whereas palpable pulses were detected in the left carotid artery and bilateral dorsalis pedis. Blood pressure measurements indicated a significant discrepancy between the right (64/37 mmHg) and left (101/47 mmHg) brachial readings, as well as elevated readings in the right (164/78 mmHg) and left (178/73 mmHg) popliteal regions. Laboratory analysis revealed an elevated erythrocyte sedimentation rate and a slightly elevated C-reactive protein level of 33 mm/h and 0.22 mg/dL, respectively. According to the American College of Rheumatology guidelines, the diagnosis of Takayasu’s arteritis requires the fulfillment of at least three of the six criteria: 1) age younger than 40 years, 2) claudication of extremities, 3) decreased pulses in the upper extremities, 4) blood pressure difference of 10 mmHg or more between arms, 5) bruits over the subclavian arteries or aorta, and 6) angiographic proof of narrowing or occlusion of the aorta or its major branches. In this case, the patient met three criteria for the diagnosis of Takayasu’s arteritis: absent pulses in the bilateral upper extremities, a 40-mmHg systolic blood pressure difference between arms, and angiographic confirmation of stenosis or occlusion of the major branches of the aorta on brain MRA.6) Subsequently, the patient was referred to the rheumatology department. Further evaluation with neck computed tomography angiography indicated complete occlusion of the right brachiocephalic trunk, left common carotid artery, and left subclavian artery, as well as stenosis and occlusion of the left brachiocephalic artery. Chest MRI revealed vascular wall hypertrophy and contrast enhancement in the descending thoracic aorta and left common carotid artery suggesting arteritis (Fig. 1). Additionally, neck ultrasound examination confirmed occlusion of the right internal carotid artery and left brachiocephalic artery as well as stenosis of the left common carotid artery. To rule out autoimmune causes, further testing including rheumatoid factor, antinuclear antibodies, antineutrophil cytoplasmic antibodies, cANCA, pANCA, and anticardiolipin antibodies was conducted, all of which yielded negative results. The patient exhibited improvement in headache after initiating immunosuppressive therapy.

jcohns-34-4-210-g1
Fig. 1. Computed tomography scan of the neck showing occlusion (arrow) of the following vessels. A: Right common carotid artery. B: Left common carotid artery. C: Left vertebral artery. D: Magnetic resonance imaging scan showing delayed wall enhancement and double ring sign (arrow) of the descending aorta, suggesting active aortitis.
Download Original Figure

During hospitalization, an otolaryngologic consultation was requested for the management of bilateral nasal obstruction that had persisted for 1 year. The patient had no history of trauma, nasal diseases, or previous nasal surgeries as well as illicit drug use, exposure to heavy metals, or smoking. Endoscopy showed a large perforation in the caudal septum, characterized by significant dry crusts along its borders, while no other malignant, inflammatory, or granulomatous changes were observed (Fig. 2A). Paranasal sinus computed tomography imaging revealed no abnormalities in the nasal cavity or paranasal sinuses but confirmed the size of the septal perforation to be 18×14 mm (Fig. 2B). After excision of the exposed bony edges of the perforation using cutting forceps, a reduction in crust formation was observed. The patient did not experience discomfort or desire for surgical correction; therefore, conservative management was pursued.

jcohns-34-4-210-g2
Fig. 2. Photos of nasal septal perforation. Caudal nasal septal perforation can be detected via nasoendoscopy (A). Paranasal sinus computed tomography scans showing nasal septal perforation in the axial view (B).
Download Original Figure

Discussion

Takayasu’s arteritis is characterized by cellular infiltration leading to vascular tunica media thickening and localized vascular smooth muscle destruction. Fibrotic reactions and hyperplasia of the tunica media and intima occur, resulting in blood vessel narrowing or stenosis.7) In the early stages before significant vascular lumen narrowing, nonspecific symptoms are more common than ischemic ones, and during this period, patients may not be aware of the disease. In the later stages, ischemic symptoms caused by arterial occlusion occur,1) and 5%–20% of patients with Takayasu’s arteritis experience neurological symptoms such as stroke, transient ischemic attacks, and visual disturbances.8)

The treatment goal for Takayasu’s arteritis is to suppress inflammatory response and maintain stable blood flow.2) Steroids are commonly used to suppress immune response; additional immunosuppressive agents such as Methotrexate can also be utilized. Joveret et al. reported that the combination of steroids and Methotrexate reduces the total amount of steroid usage and lowers the frequency of disease relapse.9) On the other hand, studies by Hoffman et al. and Spiera et al. showed no significant difference between steroid monotherapy and combination therapy.10,11) In cases where severe symptoms persist due to arterial stenosis and occlusion even after the acute inflammatory phase, surgical interventions such as stent placement, endarterectomy, bypass graft surgery, and angioplasty may be considered.2) In the present case, steroid was administrated in the acute inflammation phase, and Aspirin, Plavix, and Methotrexate were used to manage the inflammation as a maintenance therapy.

Septal perforation occurs due to various causes, and Wegener’s granulomatosis or sarcoidosis has been reported to cause granulomatous inflammation leading to septal damage and the development of septal perforation and saddle nose deformity.12) Yamasaki reported a case of septal cartilage and maxillary sinus necrosis in a patient with Wegener’s granulomatosis and Takayasu’s arteritis.13) Additionally, septal perforation can occur due to ischemic changes. Blockage of blood flow to the septum can occur due to pressure from intranasal hemostatic agents used for controlling epistaxis or vasoconstriction caused by nasal sprays or cocaine abuse. In some rheumatic diseases, such as systemic lupus erythematosus and antiphospholipid antibody syndrome, septal perforation can occur due to ischemic changes in the septum.12,14) The septal mucosa receives blood supply from the ophthalmic arteries originating from the internal carotid artery and from branches of the maxillary artery originating from the external carotid artery, with the terminal artery of the maxillary artery, called the sphenopalatine artery, supplying the major blood flow to the septal mucosa.1) If carotid artery stenosis or occlusion occurs due to Takayasu’s arteritis, ischemic changes in the septal mucosa can lead to septal perforation. Shine et al. reported a case of saddle nose deformity in a patient with Takayasu’s arteritis,15) and Akar et al. reported the first case of septal perforation in a patient with Takayasu’s arteritis, although the patient’s condition deteriorated, resulting in death following medication treatment and cerebral vascular bypass surgery.1) In the present case, the patient had stenosis of both the internal carotid arteries, but after medication treatment, the headache symptoms improved, and the patient has been undergoing treatment without any significant complications for 11 years.

Histological biopsy to confirm granulomatous inflammation or vascular inflammation would be helpful in accurately differentiating the cause of septal perforation. However, a limitation in this case is that the patient was unable to undergo septal tissue biopsy. Nevertheless, during the 11-year follow-up period, no other systemic diseases or nasal disorders occurred aside from Takayasu’s arteritis, suggesting a low possibility of alternative causes.

Septal perforation occurring in patients with Takayasu’s arteritis has been very rarely reported, and the reported cases were associated with worsening conditions leading to death following surgery for Takayasu’s arteritis or coexistence with other rheumatic diseases. Therefore, even in patients with Takayasu’s arteritis who do not exhibit severe clinical symptoms, the possibility of septal perforation should be considered in cases where other causes have been ruled out.

Acknowledgements

Not applicable.

Funding Information

Not applicable.

Conflicts of Interest

No potential conflict of interest relevant to this article was reported.

Author Contribution

Conceptualization: Heo SJ.

Data curation: Jung B, Kim JS.

Formal analysis: Jung B, Kim JS, Heo SJ.

Methodology: Jung B, Kim JS, Heo SJ.

Validation: Heo SJ.

Writing - original draft: Jung B.

Writing - review & editing: Jung B, Kim JS, Heo SJ.

Ethics Approval

The study was approved by the Institutional Review Board of the Kyungpook National University Chilgok Hospital (IRB Approval No. 2022-1227-1).

References

1.

Akar S, Dogan E, Goktay Y, Can G, Akkoc N, Sarioglu S, et al. Nasal septal perforation in a patient with Takayasu’s arteritis: a rare association. Intern Med 2009;48(17):1551-4.

2.

Larrazabal RB Jr, Chiu HHC, Magbitang-Santiago AT. Takayasu arteritis presenting as bilateral ocular ischemic syndrome. Vasc Specialist Int 2020;36(3):163-9.

3.

Chmiel JF. Septal perforation: medical aspects. Emerg Med J 2001;2(9):1-11.

4.

Gubbels SP, Barkhuizen A, Hwang PH. Head and neck manifestations of Wegener’s granulomatosis. Otolaryngol Clin North Am 2003;36(4):685-705.

5.

Bruno E, Russo S, Nucci R, Alessandrini M, Di Girolamo S. Nasal mucosal involvement in systemic lupus erythematosus: histopathologic and immunopathologic study. Int J Immunopathol Pharmacol 2000;13(1):39-42.

6.

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(8):1129-34.

7.

Hotchi M. Pathological studies on Takayasu arteritis. Heart Vessels 1992;7(Suppl 1):11-7.

8.

Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis? Rheum Dis Clin North Am 2007;33(4):777-86.

9.

Jover JA, Hernández-García C, Morado IC, Vargas E, Bañares A, Fernández-Gutiérrez B. Combined treatment of giant-cell arteritis with methotrexate and prednisone: a randomized, double-blind, placebo-controlled trial. Ann Intern Med 2001;134(2):106-14.

10.

Hoffman GS, Cid MC, Hellmann DB, Guillevin L, Stone JH, Schousboe J, et al. A multicenter, randomized, double‐blind, placebo‐controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum 2002;46(5):1309-18.

11.

Spiera RF, Mitnick HJ, Kupersmith M, Richmond M, Spiera H, Peterson MGE, et al. A prospective, double-blind, randomized, placebo controlled trial of methotrexate in the treatment of giant cell arteritis (GCA). Clin Exp Rheumatol 2001;19(5):495-501.

12.

Kridel RWH. Considerations in the etiology, treatment, and repair of septal perforations. Facial Plast Surg Clin North Am 2004;12(4):435-50.

13.

Yamasaki S, Eguchi K, Kawabe Y, Tsukada T, Nagataki S. Wegener’s granulomatosis overlapped with Takayasu arteritis. Clin Rheumatol 1996;15(3):303-6.

14.

Mascarenhas R, Tellechea O, Oliveira H, Reis JP, Cordeiro M, Migueis J. Nasal septum perforation as the presenting sign of lupus erythematosus. Dermatol Online J 2005;11(2):12.

15.

Shine NP, Hamilton S, McShane DP. Takayasu’s arteritis and saddle nose deformity: a new association. J Laryngol Otol 2006;120(1):59-62.

34권 4호 Quiz 정답 및 해설, 당첨자 발표


정답 ②

- 소음환경에서의 청취 편의성과 음성 명료성을 위하여 
디지털 신호 처리 기술이 사용되고 있는데 
그중 대표적인 것이 디지털 소음 감소(digital noise reduction, DNR) 기능과  
방향성 기술(directionality) 사용이다.

- 미국의 Food and Drug Administration(FDA)의 정의에 따르면 PSAP는 
단순히 소리를 증폭하는데 사용되며, 정상 청력을 가진 모든 연령의 
일반인에게 특정상황에서 소리를 증폭하는데에 사용되는 일반 전자제품이다. 
하지만 최근의 제품들은 경도와 중등도 난청 환자에서 보청기와 유사한 효과를 
보인다는 연구 결과를 보이기도 하였다. 아직 고도난청 환자에게 PSAP 를 
적용하는 것에 대한 효과는 증명되어 있지 않다.

- 최신 보청기에는 심박동 센서와 움직임 센서를 탑재 하여 걸음 수, 심박동변화, 
및 착용시간을 추적관리 할 수 있도록 하였고, 다른 모델에서는 움직임 센서를
기반으로 하여  낙상을 감지하여 사용자나 사용자의 가족에게 통보해 줄 수 있는 
기능을 탑재 하였다.

- 저전력 블루투스 기술을 사용할 경우 더 낮은 전력소모량으로 
더 나은 음질을 확보할 수 있다.
 

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