Case Author(s): J. Philip Moyers, MD and Farrokh Dehdashti, MD , 11/16/95 . Rating: #D4, #Q4

Diagnosis: Takayasu's Arteritis

Brief history:

This patient presents with left- sided shoulder pain.

Images:

Projection images from a lung perfusion scan

View main image(pe) in a separate image viewer

View second image(vq). Projectional images from Xenon-133 ventilation portion of VQ scan

View third image(an). Anterior projection of right PA injection, LAO projection of arch aortogram

Full history/Diagnosis is available below


Diagnosis: Takayasu's Arteritis

Full history:

37-year old Oriental woman with left shoulder pain and shortness of breath and known history of Takayasu's arteritis

Radiopharmaceutical:

17.1 mCi Xe-133 gas by inhalation and 4.4 mCi Tc-99m MAA i.v.

Findings:

Overall decreased perfusion to the entire left lung, especially the apical-posterior segment of the let upper lobe. There is also a small perfusion defect in the anterior segment of the right upper lobe (on the RPO view).

Discussion:

Takayasušs arteritis, also known as pulseless disease, is a chronic inflammatory panarteritis of unknown etiology affecting predominantly large central arteries, including the aorta (and its branches) and the pulmonary arteries. 2.6 new cases per million are diagnosed per year. This disease typically afflicts young Oriental females (age range 15-41 years). The clinical signs and symptoms may be divided in a pre-pulseless phase and a pulseless phase. The pre-pulseless phase, which may last from several months to a year, is characterized by weight loss, fevers, myalgias, arthralgias, and other nonspecific symptoms. The pulseless phase includes claudication, pulse deficits, bruits (plus other signs and symptoms of limb ischemia), and renal vascular hypertension.

The angiographic findings include both long, diffuse, and short segmental irregular stenotic areas of the major branches of the aorta near their origins. The thoracic aorta is more commonly affected than the abdominal aorta. The angiographic findings typically describe skip areas and abundant collaterals. Fusiform aortic aneurysm and ectasias are seen in 10-15%.

Followup:

Follow-up pulmonary angiogram proved Takayasu's arteritis.

Differential Diagnosis List

Takayasu's arteritis may be mistaken for pulmonary embolism as both conditions exhibit abnormal perfusion and normal ventilation on ventilation-perfusion scintigraphy.

ACR Codes and Keywords:

References and General Discussion of Ventilation Perfusion Scintigraphy (Anatomic field:Lung, Mediastinum, and Pleura, Category:Other generalized systemic disorder)

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Case number: vq012

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