Blau syndrome is a monogenic disease resulting from mutations in the pattern recognition receptor NOD2, and is phenotypically characterized by the triad of granulomatous polyarthritis, dermatitis and uveitis. This paper reviews briefly the classical clinical features of the disease, as well as more recently described extra-triad symptoms. From an ongoing prospective multicenter study, we provide new data on the natural history of Blau syndrome, focusing on functional status and visual outcome. We also present an update of the range of different NOD2 mutations found in Blau syndrome as well as recent data on morphologic and immunohistochemical characteristics of the Blau granuloma. Finally, emerging insights into pathogenic mechanisms including activation of NOD2 signal transduction, and potential biomarkers of disease activity are discussed.

Background-In a correlative functional/histopathologic study, we investigated the regional deformation characteristics of both chronic nontransmural and transmural infarctions before and after a dobutamine challenge. Methods and Results-After stenosing copper-coated stent implantation to produce circumflex artery endothelial proliferation, 18 pigs were followed up for 5 weeks. Posteuthanasia histology showed 10 to have a nontransmural and 8 a transmural infarction. Eight nonstented animals served as controls. Regional radial function was monitored by measuring ultrasound-derived peak systolic strain rates (SR SYS ) and systolic strains (⑀ SYS ) (1) before stent implantation and (2) at 5 weeks, at baseline (bs) and during an incremental dobutamine infusion. In controls, dobutamine induced a linear increase in SR SYS (dobutamine: bs, 4.8Ϯ0.4 s

These observations indicate that capsular stimulation reduces core symptoms 21 months after surgery in patients with severe, long-standing, treatment-refractory obsessive-compulsive disorder. The stimulation elicited changes in regional brain activity as measured by functional magnetic resonance imaging and positron emission tomography.

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We report a case of a 62-year-old female patient with an inflammatory reaction of the thoracic aortic wall. The diagnosis was made by computed tomographic (CT) scan, magnetic resonance imaging (MRI) and positron emission tomographic scan with 18-fluorodeoxyglucose (FDG-PET). The patient was treated with corticosteroids. The inflammatory parameters as well as FDG-uptake on PET scan returned to normal. Due to its aspecific presentation, the diagnosis of aortitis is often hard to establish. With this case the possible role of FDG-PET scan as a valuable tool in the diagnosis and monitoring of this inflammatory aortic disorder was demonstrated.