Systemic JIA as an Autoinflammatory Disease
نویسندگان
چکیده
منابع مشابه
Determing an “autoimmune” phenotype in systemic JIA
* Correspondence: [email protected] Deutsches Zentrum für Kinderund Jugendrheumatologie, GarmischPartenkirchen, Germany SoJIA-Patients 35 Observation (month) mean 69 (range 1-262) n % n % Autoantibodies pos neg 9 25,7 26 74,3 ANA 6 17,1 RF 2 5,7 ANCA 3 8,6 percentage for each group male 3 33,3 11 42,3 female 6 66,6 15 57,7 onset age (years) 6,03 3,54 relapses 4 3,6 joints at...
متن کامل[Hereditary systemic autoinflammatory diseases].
Systemic autoinflammatory diseases encompass different rare clinical entities characterized by recurrent acute inflammatory episodes secondary to a dysregulated inflammatory process. Since their first clinical descriptions, the Mendelian hereditary nature of some of them became evident, with their genetic and molecular basis being recently elucidated. There are disease-causing mutations in gene...
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Results 2 carrier for M694V and two patients who were homozygote for MEFV mutations. Both of these patients were among the most severe patients in the group. One had an excellent response to etanercept whereas the other was resistant to anti-TNF and other conventional treatments and had only a partial response to thalidomide. Although the number of severe mutations were increased in this small ...
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Introduction Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is characterized by periodic fever, cutaneous rash, conjunctivitis, lymphadenopathy, abdominal pain, myalgia, and arthralgia. It is a rare autosomal dominant disease and strongly associated with heterozygous mutations in the tumor necrosis factor (TNF) receptor super family 1A (TNFRSF1A) gene. The great diversity o...
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Systemic juvenile idiopathic arthritis (sJIA), formerly called Still's disease, is officially classified as a subset of juvenile idiopathic arthritis (JIA). Beside arthritis, it is characterized by prominent systemic features and a marked inflammatory response. Even if it is still included in the group of juvenile arthritides, sJIA is set apart from all the other forms of JIA. This disorder has...
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ژورنال
عنوان ژورنال: Inflammation and Regeneration
سال: 2011
ISSN: 1880-9693,1880-8190
DOI: 10.2492/inflammregen.31.52