Abdominal Pain as Presentation of Takayasu's Arteritis in an Adolescent Male Patient

Farhad Zamani, MD; Ramin Shakeri, MD; Omid Modiramani, MD; Reza Malekzadeh, MD

Disclosures

March 16, 2006

Case

A 16-year-old male patient was admitted to our hospital in November 2004 for investigations of a 1-year history of generalized abdominal pain, anorexia, nausea and postprandial vomiting, intermittent fever, and weight loss (12 kg).

He was hospitalized twice because of the aforementioned complaints. In the first admission (July 2004), he had been discharged with metronidazole because of giardiasis in the stool, but a definite diagnosis was not made. Due to his continuing medical problem, he was admitted to another hospital in August 2004. Because of anemia, an elevated erythrocyte sedimentation rate (ESR) (laboratory tests are shown in Table 1 ), fever of unknown origin, and a positive purified protein derivative (PPD) test, treatment with antituberculosis drugs (isoniazid, rifampin, ethambutol, and pyrazinamide) was started. Despite continuing the antituberculosis drugs for 2 months, he did not improve and medication was stopped.

In November 2004, he was admitted to our hospital with the aforementioned symptoms. Meticulous history taking revealed that he recently suffered from pain and fatigue while using his left arm.

During physical examination, discrepancy between muscle force of the right and left upper extremities and weak radial and brachial pulses in the left side were detected. Blood pressure in the right arm was: 130/80 mm Hg, whereas blood pressure in the left arm was 100/60 mm Hg; oral temperature was 37°C; pulse rate was 80/minute, and respiratory rate was 16/minute. Bruit on the carotid and subclavian and renal arteries was not heard.

Chest, heart, and abdominal examination were normal. Neurologic examination was normal except for mild weakness in the left upper extremity (proximal muscle force, 4 of 5).

Upper gastrointestinal endoscopy was done, which was normal. In chest x-ray, widening of the mediastinum due to aortic dilatation compatible with aneurysm was detected (see Figure 1). Echocardiogram was normal.

Figure 1.

Chest x-ray: widening of the mediastinum is detected.

In view of the above positive finding and suspicion of TA, magnetic resonance angiography (MRA) was performed, which revealed irregularity and aneurysm (diameter, 4.5 cm) in the descending aorta that extended to the abdominal aorta (see Figures 2 and 3).

Figure 2.

Magnetic resonance angiography: extension of irregularity and aneurysm from the descending aorta to the abdominal aorta.

Figure 3.

Magnetic resonance angiography: aneurysm of aorta.

Then we performed aortography for better evaluation of vascular involvement, which revealed long, significant stenosis of the subclavian artery after the vertebral artery, diffuse significant stenosis of the left axillary and brachial arteries, aneurysmal dilatation (fusiform) of the descending aorta from midportion down to the diaphragm level, and diffuse stenosis of the aorta after the renal artery.

In reference to the American College of Rheumatology criteria in 1990,[7] our patient had at least 4 criteria of TA (age < 40 years, decreased pulsation of 1 brachial artery, difference of > 10 mm Hg in systolic blood pressure between the arms, and arteriographic narrowing of the aorta and its primary branches) and fulfilled the definition of it. Oral prednisolone 40 mg daily was started; systemic symptoms responded to medication; ESR fell; and C-reactive protein was negative after about 6 weeks of treatment. We tapered prednisolone down gradually to 10 mg/day. The laboratory tests are shown in Table 1 .

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