If toe necrosis occurs in a woman under 40 years of age and an embolic mechanism is suspected, a specific systemic vasculitis should be searched for both clinically and radiologically according to French doctors led by Dr. Rémy Hamdan (Le CHU Dijon Bourgogne). A woman’s medical history in her 30s makes it clear why they recommend this.
The patient and her history
The patient, who had smoked for 15 years and suffered from psoriatic arthritis for 13 years, suddenly developed necrosis of the right fifth toe, according to the authors.She had also complained for several years about pain in the sacroiliac joint and in the calf, a month ago the pain had suddenly increased.
The findings
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Systolic blood pressure 146 mmHg,
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Temperature 37,
Temperature 37,
Temperature 37,4 °C and heart rate 77 beats per minute
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Tactile radial pulses, but no foot pulses
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ECG: inconspicuous
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CRP levels increased (11 mg/L)
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Hyperleukocytosis (12.3 g/L) with neutrophilia (7.95 g/L)
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Hypercholesterolemia (7.40 mmol/L)
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LDL cholesterol 5.47 mmol/L and HDL cholesterol 1.23 mmol/L
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A comprehensive metabolic panel, autoimmune tests and thrombophilia tests were normal, as were long-term Holter ECG and echocardiography.
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The ankle-arm index (ABI) of the right leg was 0.67, that of the left leg was 0.59
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Computed tomography: thickening of the left A.subclavia and the common carotid artery and high-grade stenosis of aortoiliacal bifurcation with concentric wall thickening
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Doppler ultrasound confirmed the severity of the lesion. The downstream arteries were continuous, of normal caliber and free of atheroma.
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Fluorodeoxyglucose PET inconspicuous
Systolic blood pressure 146 mmHg,
Temperature 37,
Temperature 37,
Temperature 37,4 °C and heart rate 77 beats per minute
Tactile radial pulses, but no foot pulses
ECG: inconspicuous
CRP levels increased (11 mg/L)
Hyperleukocytosis (12.3 g/L) with neutrophilia (7.95 g/L)
Hypercholesterolemia (7.40 mmol/L)
LDL cholesterol 5.47 mmol/L and HDL cholesterol 1.23 mmol/L
A comprehensive metabolic panel, autoimmune tests and thrombophilia tests were normal, as were long-term Holter ECG and echocardiography.
The ankle-arm index (ABI) of the right leg was 0.67, that of the left leg was 0.59
Computed tomography: thickening of the left A.subclavia and the common carotid artery and high-grade stenosis of aortoiliacal bifurcation with concentric wall thickening
Doppler ultrasound confirmed the severity of the lesion. The downstream arteries were continuous, of normal caliber and free of atheroma.
Fluorodeoxyglucose PET inconspicuous
Diagnosis, therapy and course
As the authors report, they made the diagnosis of Takayasu’s arteritis (TA) in the chronic phase based on gender, age, clinical presentation, typical radiological involvement and negative PET findings. The isolated involvement of the fifth toe was, according to them, compatible with an embolic event. The therapy consisted of nicotine cessation, infliximab, , enoxaparin and atorvastatin. According to Rémy Hamdan and his colleagues, toe lesions receded, and a CT scan showed an unchanged aortoiliacal lesion. During the follow-up examinations, the woman continued to complain of pain in the lower limbs, so that an endovascular reconstruction of the aortic bifurcation had been performed.The ankle-arm index had then normailized, the pain had decreased.
Discussion
Takayasu’s arteritis is a systemic vasculitis of large vessels, which preferentially affects the aorta and the large arteries extending from the aorta, explains Professor Dr. med. Bernhard Hellmich (Vasculitis Center South, Medius Kliniken – Academic Teaching Hospital of the University of Tübingen). In the course of the disease, vasculitis also leads to a remodeling of the affected vessel walls via myofibroblast proliferation. As a consequence of the structural changes in the vessel walls, vascular stenosis would occur and, in about a quarter of those affected, aneurysmal vasodilation would occur. According to Hellmich, the structural vascular changes lead to functional limitations in most patients; the mortality of TA is increased despite immunosuppressive therapy. However, with an incidence of about 1 new case per 1 million inhabitants per year, TA is a rare disease in Europe (unlike in Asia).It mainly affects young adults, typically in the second and third decade of life.
According to Hellmich, symptoms and clinical findings that indicate inflammatory activity of Takayasu arteritis according to the EULAR (European League Against Rheumaism) expert consensus are:
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New or worsened claudication of the extremities
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Constitutional symptoms (such as weight loss>2 kg, mild fever, fatigue, night sweats)
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Myalgia, Arthralgia, Arthritis
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Severe abdominal pain
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Stroke, seizures (not hypertension-related), syncope, dizziness
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Myocardial infarction, angina pectoris
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Acute visual symptoms such as amaurosis fugax or diplopia
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Hypertension (>140/90 mm Hg), new loss of pulse, pulse difference and carotidynia.
Paresis of the extremities
New or worsened claudication of the extremities
Constitutional symptoms (such as weight loss>2 kg, mild fever, fatigue, night sweats)
Myalgia, Arthralgia, Arthritis
Severe abdominal pain
Stroke, seizures (not hypertension-related), syncope, dizziness
Paresis of the extremities
Myocardial infarction, angina pectoris
Acute visual symptoms such as amaurosis fugax or diplopia
Hypertension (>140/90 mm Hg), new loss of pulse, pulse difference and carotidynia.
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