Teaching Case

A 30-year-old Ecuadorian woman came to the emergency service with acute dyspnoea and chest pain. She referred weight loss and self-limited episodes of chest pain for the last month.

Physical examination showed bilateral crackles, 33% saturation and 220/120 hypertension. A diagnosis of acute pulmonary oedema was made.

On the CT images there is a concentric wall thickening of the thoracic and abdominal aorta. The lumen of the aorta is moderately reduced at some points. The abdominal aorta is the most affected segment. The origins of the coeliac axis, superior mesenteric artery and renal arteries are highly stenosed. Distally these arteries recover the normal calibre.

In the pre-contrast CT the thickened wall has a high attenuation level (54 HU) and it shows uniform enhancement after the contrast administration (100 HU).

The pulmonary parenchyma shows areas of ground-glass opacity and dense consolidations in dependent lung caused by acute pulmonary oedema.

Takayasu arteritis is an idiopathic chronic vasculitis affecting medium and large size arteries [1-4]. It is an uncommon disease (1-3 cases per million) with the greatest prevalence in Asians, followed by Hispanics [5, 6]. It is more frequent in young women between 10 and 40 years of age.

The classic involvement of the aortic arch and its branches is more frequent in the Japanese population, whereas in India, Thailand and China the abdominal aorta and renal arteries are the most affected vessels [5, 6]. Knowledge of the individual variability of the disease prevents misdiagnosis. [5, 7]

Other arteries may also be affected such as pulmonary arteries and coronary territory [1-6]. The abdominal aorta and pulmonary arteries are involved in approximately 50 percent of patients. [1, 7]

The inflammatory process causes thickening of the walls and narrowing of the lumen of the affected vessels. Total occlusion and even dilatation of involved portions of the arteries may occur later on. [1, 3, 4]

This disease has been divided into an early, “prepulseless” systemic phase, with constitutional non-specific symptoms and a late occlusive phase. Most patients are diagnosed in the late phase. [3, 4, 7]

In the occlusive phase there is a wide diversity of symptoms depending on the affected segment: subclavian steal syndrome due to stenosis of the subclavian artery proximal to the origin of the vertebral artery, pulse asymmetry in extremities and claudication, pulmonary manifestations such as chest pain, dyspnoea and pulmonary hypertension, mesenteric artery ischaemia, reno-vascular hypertension due to narrowing of the renal artery, angina pectoris and myocardial infarction when there is coronary arteritis, etc. [1, 3]

In most cases, the diagnosis is based upon imaging by CT scan, magnetic resonance imaging or conventional angiography. CT and MRI are the diagnostic techniques of choice [4, 7]. Arteriography may also allow therapeutic intervention (angioplasty, stenting)
High-dose corticosteroids is the treatment of choice although the response in uncertain [2, 5]

After a fortnight with high-dose of corticosteroids our patient was completely asymptomatic.