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

Abstract

We describe a boy with chronic abdominal pain, nausea and vomiting, and weight loss. The imaging was compatible with Takayasu's arteritis. Chronic mesenteric ischemia was the etiology of the patient's symptoms.

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Background

Takayasu's arteritis (TA) is a chronic vasculitis of unknown etiology. It mainly affects young women in the second and third decades of life.[1] It has a worldwide distribution, with the greatest prevalence in Asians.[2]

It primarily affects the aorta and its branches. Systemic symptoms, including fatigue, weight loss, and low-grade fever, are common in the early phase of disease. Vascular symptoms are rare at presentation, but with progression of disease, evidence of vascular involvement and insufficiency becomes clinically apparent due to dilation, narrowing, or occlusion of the proximal or distal branches of the aorta.

Subclavian artery involvement is common, and a stenotic lesion proximal to the origin of the vertebral artery can lead to neurologic symptoms.[3] Other symptoms include arthralgias; myalgia; skin lesions; symptoms related to pulmonary, carotid, vertebral, and coronary artery involvement; abdominal pain; and nausea, vomiting, and diarrhea that may result from mesenteric artery ischemia, which is rare in TA.[4–6]

We present a 16-year-old boy who first complained of recurrent abdominal pain and vomiting and was finally diagnosed with TA.

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.

Table 1.

Lab Tests

	June 2004	August 2004	Nov 2004	After Treatment
WBC	7400/ul	8000/ul	8400/ul
Hb	9.7 g/dl	8.5 g/dl	8.5 g/dl	12.7 g/dl
MCV	71.5 fl	70 fl	70 fl	75.1 fl
MCH	22.1 pg	22 pg	22.2 pg	23.0 pg
MCHC	30%	30.9%	30.9%	30.7%
Platelet	500000/ul	450000/ul	480000/ul	366000/ul
ESR	130	140	130	19
Urinanalysis	Normal	Normal	Normal
AST	13	15	13	15
ALT	20	20	25	16
Bun	10	14	14	-
Creatinine	0.6	0.6	0.4	-
CRP	-	Positive	Positive	-
ANA	-	Negative	-
ANCA	-	Negative	-
PPD Test	-	14 mm	-

WBC = white blood cell; Hb = hemoglobin; MCV = mean corpuscular volume; MCH = mean corpuscular hemoglobin; MCHC = mean corpuscular hemoglobin concentration; ESR = erythrocyte sedimentation rate; AST = aspartate aminotransferase; ALT = alanine aminotransferase; CRP = C-reactive protein; ANA = antinuclear antibody; ANCA = antineutrophil cytoplasmic antibody; PPD = purified protein derivative

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.

Discussion

TA is a rare, nonspecific obliterative arteritis of unknown origin that involves a large or a medium and large vessel with arteritis (Chapel Hill classification); it occurs predominantly in young, Asian females of childbearing age.[2] Reports of the disease in male patients are very rare. The pathologic process involves all layers of the arterial wall. As with many rare diseases, delay in diagnosis is an important issue for patients with TA. TA may present a wide variety of signs and symptoms depending on the vessel that is affected.[8]

It is infrequently associated with chronic abdominal pain and vomiting because mesenteric ischemia is a rare manifestation of the disease. In a recently published article, in a period of 27 years from the 33 patients with descending thoracic or thoracoabdominal aortic aneurysm in association with TA, the revealing symptom of just 7 patients was thoracic or abdominal pain.[9]

This report emphasizes the importance of ensuring a thorough systemic evaluation in individuals who are experiencing abdominal pain with systemic symptoms. TA should be considered as one of the underlying diseases when a patient develops protean manifestations of the gastrointestinal tract, including chronic and recurrent abdominal pain and constitutional symptoms even in male patients.