A 26-year woman comes to the office complaining about pain and numbness in her upper arms, exacerbated by strenuous activity. She also reports a 12-pound weight loss, joint pain and fatigue in the past six months. She has no smoking history and denies any use of illicit drugs. Her blood pressure is 143/89 mmHg in the right arm and 87/56 mmHg in the left. Her temperature is 37.6°C (99.68°F) and her heart rate is 90 bpm. Auscultation of the heart shows no murmurs and lungs are clear. The brachial pulses in her left arm are decreased compared to her right arm. No skin or mucosa lesions are identified, and the abdominal examination is unremarkable. The results of complete blood count and renal function are normal, except for mild anemia and an erythrocyte sedimentation rate (ESR) of 56 mm/hr.
The correct answer is: E. Takayasu arteritis
Takayasu arteritis is a rare, systemic, inflammatory large-vessel vasculitis of unknown etiology that most commonly affects women of childbearing age. It affects mainly the aorta, its major branches and the pulmonary artery. The mean age at diagnosis is between 25 and 30 years. The majority of patients will demonstrate a relapsing/remitting or progressive course.
Clinical presentations vary from non-specific constitutional symptoms (fatigue, joint pain, fever, weight loss, increased ESR) without clinical or radiographical evidence of arterial occlusive disease (pre-pulseless stage) to symptoms attributed to arterial stenosis, aneurysm formation and occlusion (pulseless stage). Patients can present with a variety of symptoms depending on the affected vascular territory. The involvement of the subclavian arteries may cause upper extremity claudication, digital ischemia/gangrene, interarm blood pressure discrepancy >10mm Hg (as in this case). Additionally, involvement of the aorta can be complicated by aneurysm formation, aortic regurgitation due to aortic dilatation, and aortic stenosis. Any of the above-mentioned arterial lesions can present with diminished or absent pulses and/or synchronous vascular bruits.
Kawasaki disease causes inflammation in the walls of medium-sized arteries throughout the body and primarily affects children. The inflammation tends to affect the coronary arteries and not the major branches of the aortic arch. For the diagnosis of Kawasaki disease, the patient must have fever ≥5 days and ≥4 additional of the following criteria: >1.5 cm cervical lymph node, polymorphous rash, edema of hands and feet, conjunctivitis and mucosal changes. (Answer A). Kawasaki disease is not the correct answer.
Patients with coarctation of the aorta commonly exhibit higher blood pressure (hypertension) measurements in their arms than in their legs. Moreover, in those patients the pulses of lower extremities are often decreased and delayed compared to hands' pulses. (Answer B)
Although patients with dissection of the aorta may present with interarm blood pressure discrepancy >10mm Hg, this finding is usually accompanied with sudden onset severe chest or upper back pain, often described as a tearing, ripping or shearing sensation, which is not present in this case. (Answer C)
In acute pericarditis the most common symptom is sharp, stabbing chest pain behind the sternum or referral pain located in the left hemithorax. It often intensifies with coughing, lying down or deep inspiration. Sitting up and leaning forward usually reliefs the pain. Heart sounds are distant and friction rub may be heard. None of the above symptoms and signs are identified in this case. (Answer D)
Granulomatosis with polyangiitis affects the nose, sinuses, throat, lungs and kidneys. This patient has no ulcers in her oral or nasal mucosa. She has no respiratory symptoms and renal function is normal, making the diagnosis of Granulomatosis with polyangiitis very unlikely. (Answer F)
Takayasu arteritis is a chronic, idiopathic, inflammatory disease that primarily affects large vessels, such as the aorta and its main branches. The clinical features usually reflect limb or organ ischemia resulting from gradual stenosis of the involved arteries.
- Kim ESH, Beckman J. Takayasu arteritis: challenges in diagnosis and management. Heart 2018;104:558-65.
- Kerr GS, Hallahan CW, Giordano J, et al, Takayasu Arteritis. Ann Intern Med 1994;120:919-29.