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aneurysms and autoinflammatory

Perrine Dusser, Isabelle Koné-Paut
Kawasaki disease (KD) is an acute inflammatory vasculitis occurring in young children before 5 years and representing at this age, the main cause of acquired heart disease. A single infusion of 2 g/kg of intravenous immunoglobulins along with aspirin has reduced the frequency of coronary artery aneurysms from 25 to 5%. However, 10-20% of patients do not respond to standard treatment and have an increased risk of cardiac complications and death. The development of more potent therapeutic approaches of KD is an urgent need...
2017: Frontiers in Pharmacology
Takahito Ninomiya, Hidetoshi Takada, Yusaku Nagatomo, Etsuro Nanishi, Hazumu Nagata, Kenichiro Yamamura, Takehiko Doi, Kazuyuki Ikeda, Toshiro Hara
Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) is one of the autoinflammatory diseases of unknown etiology characterized by regularly recurrent fever episodes with attacks lasting 3-6 days every 3-8 weeks associated with at least one of the three cardinal clinical signs: aphthous stomatitis, pharyngitis, and cervical adenitis. Kawasaki disease (KD) is an acute, self-limited systemic vasculitis that occurs predominantly in infants and young children. In most KD patients, i...
December 2013: Pediatrics International: Official Journal of the Japan Pediatric Society
I Kötter, T Xenitidis, G Fierlbeck, S Schanz, A Melms, M Horger, U Ernemann, C Deuter
Behçet's disease is a systemic disorder with the histopathological correlate of leukocytoclastic vasculitis. Pathogenetically, besides a strong genetic component participation of the innate immune system and an autoinflammatory component are discussed. The disease is most common in countries along the former silk route but in Germany the disease is rare (prevalence approximately 0.6/100,000). Oral aphthous ulcers are the main symptom, followed by skin manifestations, genital ulcers and oligoarthritis of large joints...
October 2012: Zeitschrift Für Rheumatologie
Kikuo Isoda, Manabu Kitagaki, Tomiharu Niida, Harumi Kondo, Osamu Matsubara, Makoto Kikuchi, Fumitaka Ohsuzu, Takeshi Adachi
Femoral artery aneurysms (FAAs) are very rare, and their natural history is not well understood. In this study, we sought to analyze the pathogenesis of inflammatory FAAs in interleukin-1 receptor antagonist-deficient (IL-1Ra(-/-)) B6 mice. Systolic arterial pressures and plasma lipid levels of IL-1Ra(-/-) mice and wild-type (WT) mice did not differ significantly. However, IL-1Ra(-/-) mice spontaneously developed fusiform FAAs. Real-time PCR of 9-month-old IL-1Ra(-/-) mice revealed significantly increased mRNA levels of IL-1β (6...
March 2012: American Journal of Pathology
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