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Horner’s syndrome as an initial manifestation of Takayasu’s arteritis
  1. J-W Chung1,
  2. Y-J Suh1,
  3. J-H Choi1,
  4. H-S Park1,
  5. J-H Won2,
  6. C-H Suh1
  1. 1Department of Allergy-Rheumatology, Ajou University School of Medicine, Suwon, South Korea
  2. 2Department of Radiology, Ajou University School of Medicine, Suwon, South Korea
  1. Correspondence to:
    Dr C-H Suh, Department of Allergy-Rheumatology, Ajou University School of Medicine, Paldalgu Woncheondong San-5, Suwon, South Korea 442–721;
    chsuh{at}ajou.ac.kr

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Takayasu’s arteritis (TA) is a granulomatous vasculitis of the large arteries. It typically produces segmental arterial narrowing and occlusion, but aneurysm formation is uncommon.1 Subclavian artery aneurysm presenting with Horner’s syndrome is very rare.2 We report on a patient with Horner’s syndrome caused by subclavian artery aneurysm in TA.

CASE REPORT

A 37 year old Korean man was admitted because of right side ptosis and anhidrosis. He had visited the ophthalmology department three weeks previously. Initially, his margin reflex distance 1 (MRD1) was 1.5 mm (right) and 3.0 mm (left). The neostigmine test was performed, but the MRD1 did not change. After administration of phenylephrine 1 drop, however, MRD1 was improved to 3.0 mm in the right eye. Horner’s syndrome was diagnosed. He had no trauma history. He did not complain of any claudication, pain, or dizziness. His blood pressure was 110/70 mm Hg in both arms and pulse rate 80 beats/min. Complete blood counts, serum protein, and urine analysis were normal. Antinuclear antibody and rheumatoid factor were negative. Erythrocyte sedimentation rate (ESR) at 70 mm/1st h and C reactive protein at 25 mg/l were raised.

Chest computed tomography (CT) showed a 4 cm sized aneurysmal dilation of the right subclavian artery at the thoracic inlet level with mural thrombi (fig 1A) and the proximal part of the right vertebral artery. Angiography also showed aneurysmal dilation (fig 2A). In this case, both the involvement of the subclavian and vertebral artery and the elimination of other possible causes in a young man led to the diagnosis of TA.

Figure 1

(A) CT image of the chest, showing a 4 cm sized focal aneurysm dilation of the right subclavian artery at the thoracic inlet level with mural thrombi. (B) After three months, the follow up CT image of the chest, showing the markedly decreased size of the subclavian and vertebral artery aneurysms and no evidence of newly developed lesions.

Figure 2

(A) Angiographic appearance of the right subclavian and proximal part of the right vertebral artery aneurysm. (B) Angiographic appearance of stent-graft in the right subclavian artery.

After CT and angiography, daily treatment with 60 mg prednisolone was started owing to high inflammatory markers. We inserted a stent-graft in the right subclavian artery (fig 2B) and coiling in the right vertebral artery after the inflammation resolved. Follow up CT showed well enhanced lumen of the subclavian artery stent and no evidence of enhancement of the aneurysm. He was discharged with slightly improved ptosis on a regimen of prednisolone 60 mg daily. Anhidrosis, however, was not improved. The prednisolone dosage was slowly tapered. After three months, he took daily 12.5 mg prednisolone and 100 mg azathioprine, and follow up CT showed the markedly decreased size (1 cm) of the subclavian artery aneurysm (fig 1B).

DISCUSSION

The clinical manifestation of Horner’s syndrome is unilateral miosis, ptosis, and ipsilateral anhidrosis, and major causes include neoplasms, cysts, or inflammatory diseases. At initial evaluation, chest CT was done to evaluate the possibility of malignancy in the thorax. Subclavian and vertebral artery aneurysms were disclosed.

Takayasu’s arteritis is a chronic granulomatous inflammatory vasculitis. A “triphasic” pattern has been described.1 Phase I is characterised by non-specific systemic complaints. Phase II is dominated by vessel pain due to vessel inflammation. Phase III refers to the fibrotic stage, in which bruits and ischaemia predominate. In some series about 16% of patients had constitutional symptoms, and 10% were asymptomatic.3 In addition, both inflammatory and fibrotic changes may be present simultaneously. Aneurysms in TA are uncommon and the incidence varies from 4.9% to 31.9%.4,5 The most common site is the aorta, followed by the subclavian artery. In our patient there were no constitutional symptoms, bruit, or ischaemic symptoms. No laboratory test is specific for TA. The ESR is often used to assess disease activity.6 Computed tomography of the chest and abdomen may be helpful in evaluating large arteries, and serial examinations may thus be useful.7

For active TA, glucocorticoids are the first treatment. Corticosteroids effectively suppress the systemic symptoms and usually arrest progression. The raised acute phase reactants also return to normal.8 Arterial stenosis may reverse and ischaemic symptoms may improve in early cases. We started 60 mg of daily prednisolone and gradually reduced the dose when the inflammatory process improved. Open surgical repair was the definitive treatment for vascular lesions, such as aneurysms. However, the success rate has not been high because a false aneurysm often occurs. To avoid surgical complications, endovascular methods, such as insertion of a stent-graft, have been recommended because they are less invasive. Recently, stent-graft placement has been indicated for the treatment of aneurysms.9 The technical success rate of stent-graft insertion is >90%. In our case, aneurysm size decreased rapidly after stent-graft insertion.

This is, to our knowledge, the first report of a patient with TA manifested by Horner’s syndrome. When Horner’s syndrome is diagnosed, a common cause is neoplasms. However, Horner’s syndrome may be the first symptom of TA.

REFERENCES

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