Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation of our cases was 11 years (range 8-15). Three ...

BACKGROUND Although there are various published epidemiological studies regarding Takayasu's arteritis (TA), none have analyzed the influence of gender on the clinical and laboratory manifestations or vascular alterations at disease onset. OBJECTIVES To analyze the influence of gender on clinical and laboratory manifestations and variations in vascular imaging at TA onset. METHODS A retrosp...

We describe a patient with a focal abdominal aortic stenosis without renovascular stenosis manifested as severe hypertension. Although hypertension and unilateral or bilateral renal artery stenosis are common in abdominal aortic stenosis, patients presenting with hypertension as the sole clinical feature without renal artery stenosis are rare. A 12-year-old boy was admitted to our department fo...

BACKGROUND Inflammatory vasculopathies, ranging from the vasculitides (Takayasu arteritis, giant cell arteritis, and polyarteritis nodosa) to atherosclerosis, display remarkable target tissue tropisms for selected vascular beds. Molecular mechanisms directing wall inflammation to restricted anatomic sites within the vascular tree are not understood. We have examined the ability of 6 different h...

Abstract Blau syndrome (BS) is a rare, autosomal dominant monogenic autoinflammatory disease, usually presenting as triad of symptoms (granulomatous dermatitis, uveitis, and nonerosive arthritis) caused by gain-of-function mutations in the nucleotide oligomerization domain 2 (NOD2) gene. However, very few reports children copresence BS with large vessel vasculitis exist. We hereby describe case...