Chest pain is one of the most frequent patient’s complaints. The commonest underlying causes are well known, but, sometimes, in some clinical scenarios, it is necessary to consider other diagnoses. We report a case of a 68-year-old Caucasian male, chronically hypertensive, who complained of recurrent episodes of chest pain and fever with elevated acute phase reactants. The first investigation was negative for some of the most likely diagnosis and he quickly improved with anti-inflammatory drugs. Over a few months, his symptoms continued to recur periodically, his hypertension was aggravated, and he developed headaches and lower limbs claudication. After a temporal artery biopsy that was negative for vasculitis, he underwent a positron emission tomography suggestive of Takayasu Arteritis. Takayasu Arteritis is a rare chronic granulomatous vasculitis of the aorta and its first-order branches affecting mostly females up to 50 years old. Chest pain is experienced by >40% of the patients and results from the inflammation of the aorta, pulmonary artery, or coronaries.
Chest pain is one of the most frequent symptoms driving patients to a physician’s practice or to the hospital’s emergency department. Although the prevalence of each aetiology differs according to the clinical setting, the underlying causes of chest pain are more commonly due to gastrointestinal, cardiac, chest wall/musculoskeletal, respiratory/pulmonary, and psychiatric disorders [
A 68-year-old Caucasian male presented to the emergency department (ER) with a 6-day history of intense retrosternal pain, radiating to the dorsum and left arm, plus fever. He also had a history of high blood pressure (HBP), medicated with olmesartan 20 mg id and headaches. On admission, he was hypertensive (162/81 mmHg) and febrile (38.2°C) without any other abnormalities at physical examination. His chest X-ray and electrocardiogram were normal. Blood analysis revealed elevated erythrocyte sedimentation rate (ESR, 81 mm) and C-reactive protein (CRP, 9.2 mg/dL) and normal myocardial necrosis markers. A transthoracic echocardiogram (TTE) was performed showing a mild pericardial thickening. Acute pericarditis was assumed and the patient was discharged with a nonsteroidal anti-inflammatory drug (NSAID).
The patient returned to the ER 2 days later with the same complaints. He maintained high acute phase reactants (APR) (ESR 82 mm, CRP 11.1 mg/dL), without other physical, laboratorial, or radiological changes, and he was then admitted to the Internal Medicine Service for further investigation. Through several virologic and serologic markers, we excluded tuberculosis, syphilis, infection by
In the meantime, the patient developed recurrent episodes of chest pain, claudication of the lower limbs, headaches, and HBP aggravation which imposed prescription reinforcement by the general practitioner (olmesartan + hydrochlorothiazide).
Six months later, he returned to the ER with a 12-day history of fever and severe headaches. Once again laboratory analysis showed not only elevated APR (ESR 120 mm, CRP 11.4 mg/dL), but also normochromic normocytic anaemia (haemoglobin 11.6 g/dL) and hypoalbuminemia (albumin 3.0 g/dL). The chest X-ray, TTE, and cerebral computed tomography were normal. The patient was admitted to the Internal Medicine Service for further investigation and underwent a temporal artery biopsy, which was negative for vasculitis. We then decided to perform a positron emission tomography (PET) that showed an increased uptake of 18F-fluorodeoxyglucose (FDG) at the subclavian, carotid, humeral, vertebral, and femoral arteries and less intensively at the ascending and descending aorta, suggestive of Takayasu Arteritis (TA) (Figure
PET scan showing increased uptake of 18F-FDG at the subclavian, carotid, humeral, vertebral, and femoral arteries and less intensively at the ascending and descending aorta, a pattern suggestive of TA. PET: positron emission tomography. 18F-FDG: 18F-fluorodeoxyglucose. TA: Takayasu Arteritis.
Arteriography showing diffuse areas of narrowing and dilation of the abdominal aorta (a) and significant dilation of the left common iliac artery (b).
The patient was reassessed on an outpatient basis 1 month later, free of symptoms, with stabilized BP and normalization of the APR and haemoglobin. By the end of the first year of treatment, he repeated PET that showed radiologic improvement but still metabolic activity of 18F-FDG (Figure
Sequential PET scan images. (a) One-year follow-up: medium intensity uptake of 18F-FDG at the subclavian, carotid, and femoral arteries. (b) Two-year follow-up: medium intensity uptake of 18F-FDG at the femoral and right external iliac. (c) Three-year follow-up: absence of 18F-FDG uptake. PET: positron emission tomography. 18F-FDG: 18F-fluorodeoxyglucose.
TA is a relatively rare chronic idiopathic granulomatous large vessel vasculitis (LVV) affecting the aorta and its first-order branches, with an estimated prevalence of 2.6/1,000,000 persons in the United States and 1.26/1,000,000 persons in northern Europe [
Symptoms resulting from systemic inflammation, such as fever, weight loss, fatigue, malaise, arthralgias, or myalgias, are common in the early stage of the disease and may represent the systemic effects of cytokines [
Vascular symptoms and signs are rare at presentation and reflect the affected arterial territories. Chest pain is experienced by >40% of the patients and results from the inflammation at the aortic arch or root level (affected in 35% of the patients), pulmonary artery (10–40%), or the coronaries (<10%) [
Interestingly, far from the commonest scenario, our patient is a 68-year-old male. He shows both systemic and vascular symptoms. His chest pain was most likely related to the involvement of the ascending aorta and his multiple and different symptoms reflect the disseminated character of his disease.
Laboratory changes reflect the inflammatory process and include elevated ESR and CRP, normochromic normocytic anemia, and hypoalbuminemia [
The diagnosis of TA is usually considered upon suggestive clinical features and imaging of the arterial tree by arteriography, magnetic resonance imaging (MRI), or computed tomography (CT) that demonstrate the characteristic pattern of irregular vessel walls, stenosis, poststenotic dilation, aneurysm formation, occlusion, and evidence of increased collateral circulation [
Several disorders, including many forms of vasculitis, must be distinguished from TA. One of the most important and difficult differential diagnoses is Giant Cell Arteritis (GCA), another granulomatous LVV that characteristically involves one or more branches of the carotid artery, particularly the temporal one, but it can also affect arteries in multiple locations and occurs almost exclusively in individuals older than 40–50 years. In fact, the age of onset of the disease is one of the most discriminatory characteristics between the two pathologies [
In this case, considering the patient’s initial complaints of chest pain and fever, we first thought of a myopericardial syndrome, but the electrocardiogram, TTE, and myocardial necrosis markers were normal. Although less likely, the hypothesis of an acute coronary syndrome was ruled out for the same reasons. We also considered a parenchymal/vascular pulmonary disease, but the atypical chest pain, normal chest X-ray, and normal leucogram made pneumonia improbable and the patient also had a low clinical likelihood for pulmonary embolism and negative d-dimers. The diagnosis of esophagitis was very unlikely, especially in an immunocompetent patient and no evidence existed to support a chest wall/musculoskeletal origin. During the first hospitalization, we have excluded many infectious aetiologies and the autoantibody tests were negative. The episodic character of the symptoms along with the new ones (headaches and limbs claudication) and the laboratorial changes (elevated APR, normochromic normocytic anaemia, and hypoalbuminemia) raised the suspicion for a systemic disease such as a vasculitis, in particular medium and large vessels one. Considering his age and headaches complaints, we first thought of GCA, and therefore we performed a temporal artery biopsy, which was negative for vasculitis. Once again, because his several and varied symptoms suggested a systemic vasculitis, we then decided to go for a broader test, thence the option for the PET scan that led us to the diagnosis of TA. We opted to keep PET for imaging follow-up and we consider our series of sequential images to be highly illustrative of the important role this image modality might play both for TA diagnosis and for follow-up.
In the presence of active disease, the initial standard treatment of TA is with high doses of prednisolone (1 mg/kg/day) or its equivalents for about a month, which are then gradually tapered until discontinuation. Glucocorticoids induce remission in about 60% of the patients, but relapses do occur in the majority (>50%) during steroid taper. In these cases, or when there is a need to counteract the side effects of steroids, a conventional immunosuppressive agent is added, usually methotrexate (25 mg/week). When these agents or a combination of these agents remain ineffective, or are not tolerated, biologic agents may be tried out [
TA is considered to be a serious disease with a chronic relapsing-remitting course causative of significant morbidity and disability. The long-term outcome of patients with TA has varied widely between studies, with a 5-year mortality rate ranging from 0 to 35% [
We believe this case highlights the importance of considering less common diagnosis when we are dealing with unexplained symptoms, especially after a relatively exhaustive first line investigation. When the clinical context strongly suggests a chronic, persistent, and inflammatory process, autoimmune aetiology must be considered.
The authors declare that there is no conflict of interests regarding the publication of this paper.