1 nov. Request PDF on ResearchGate | Maladie de Takayasu | 59) 2 Service de néphrologie et médecine interne, Hôpital de Valenciennes (59). La maladie de Takayasu est une aortoartérite aspécifique qui atteint avant tout la femme jeune et parfois l’enfant. Le traitement à la phase inflammatoire initiale. La revue de médecine interne – Vol. 30 – N° S4 – p. – L’imagerie dans la maladie de Takayasu – EM|consulte.
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Takayasu’s arteritis also known as Takayasu’s ee”aortic arch syndrome,” “nonspecific aortoarteritis,” and “pulseless disease” : It mainly affects the aorta the main blood vessel leaving the heart and its branches, as well as the pulmonary arteries.
Females are about 8—9 times more likely to be affected than males. Those with the disease often notice symptoms between 15 and 30 years of age. In the Western worldatherosclerosis is a more frequent cause of obstruction of the ds arch vessels than Takayasu’s arteritis.
Takayasu’s arteritis is similar to other forms of vasculitis, including giant cell arteritis which typically affects older individuals. Some people develop an initial “inflammatory phase” characterized by systemic illness with signs and symptoms of malaisefevernight sweatsweight lossjoint painfatigueand fainting. Fainting may result from subclavian steal syndrome or carotid sinus hypersensitivity. The initial “inflammatory phase” is often followed by a secondary “pulseless phase”.
Of note is the function of renal artery stenosis in the causation of high blood pressure: Normally perfused kidneys produce a proportionate amount of a substance called renin. Stenosis of the renal arteries causes hypoperfusion decreased blood flow of the juxtaglomerular apparatusresulting in exaggerated secretion of renin, and high blood levels of aldosteroneeventually leading to water and salt retention and high blood pressure.
The neurological symptoms of the disease vary depending on the degree; the nature of the blood vessel obstruction; and can range from lightheadedness to seizures in severe cases. One rare, important feature of the Takayasu’s arteritis is ocular involvement in form of visual field defects, vision loss, or retinal hemorrhage.
In the late stage, weakness of the arterial walls may give rise to localized aneurysms. As with all aneurysms, the possibility of rupture and vascular bleeding is existent and requires monitoring. In view of the chronic process and good collateral development, Raynaud’s phenomenon or digital gangrene are very rare in Takayasu arteritis.
A rare complication of this condition are coronary artery aneurysms. Although the cause of Takayasu arteritis is unknown, the condition is characterized by segmental and patchy granulomatous inflammation of the aorta and its major derivative branches. This inflammation leads to arterial stenosisthrombosisand aneurysms. A large collaborative study uncovered multiple additional susceptibility loci for this disease, increasing its number of genetic loci to five risk loci across the genome.
Additional genetic variants and the classical HLA alleles were imputed and analyzed. Diagnosis is based on the demonstration of vascular lesions in large and middle-sized vessels on angiography, CT scanmagnetic resonance angiography or FDG PET.
Contrast angiography has been the gold standard. The earliest detectable lesion is a local narrowing or irregularity of the lumen.
This may develop into stenosis and occlusion. The characteristic finding is the presence of “skip lesions,” where stenosis or aneurysms alternate with normal vessels. Angiography provides information on vessel anatomy and patency but does not provide information on the degree of inflammation in the wall.
The age at onset helps to differentiate Takayasu’s arteritis from other types of large vessel vasculitis.
La maladie de Takayasu : à propos de 29 cas observés en médecine interne – EM|consulte
The usual starting dose is approximately 1 milligram per kilogram of body weight per day for most people, this is approximately 60 milligrams a day. Because of the significant side effects of long-term high-dose prednisone use, the starting dose is tapered over several weeks to a dose which controls symptoms while limiting malxdie side effects of steroids.
Promising results are achieved with mycophenolate and tocilizumab . If treatment is not kept to a high standard, long-term damage or death can occur. For patients who do not respond to steroids may require revascularization, either via vascular bypass or angioplasty and stenting. Outcomes following revascularization vary depending on the severity of the underlying disease . Two Japanese physicians at the same meeting Drs.
Onishi and Kagoshima reported similar eye findings in individuals whose wrist pulses were absent.
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It is now known that the blood vessel malformations that occur in the retina are an angiogenic response to the arterial narrowings in the neck and that the absence of pulses noted in some people occurs because of narrowings of the blood vessels to the arms.
The eye findings described by Takayasu are rarely seen in patients from North America and British Columbia. From Wikipedia, the free encyclopedia.
Andrews’ Diseases of the Skin: Archived from the original on Journal of General Internal Medicine. Frontiers in Cardiovascular Medicine. Mesut; Guthridge, Joel M. American Journal of Human Genetics.
Circ Genom Precis Med 11 tzkayasu Clinical and statistical analyses of related prognostic factors” PDF. A case with peculiar changes of the central retinal vessels.
Acta Societatis ophthalmologicae JaponicaeTokyo Systemic vasculitis M30—M31 Takayasu’s arteritis Giant-cell arteritis. Acute hemorrhagic edema of infancy Cryoglobulinemic vasculitis Bullous small vessel vasculitis Cutaneous small-vessel vasculitis. Goodpasture’s syndrome Sneddon’s syndrome. Retrieved from ” https: Vascular diseases Vascular-related cutaneous conditions Steroid-responsive inflammatory conditions Systemic connective tissue disorders. Infobox medical condition new All articles with unsourced statements Articles with unsourced statements from February Wikipedia articles with NDL identifiers.