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  • Hereditary systemic autoinflammatory diseases and Schnitzler's syndrome [Elektronski vir]
    Kačar, Mark ; Pathak, Shelly ; Savic, Sinisa
    The systemic autoinflammatory diseases are disorders of the innate immune system distinguished by severe inflammation resulting from dysregulation of the innate immune system. Hereditary fever ... syndromes, such as FMF, TNF receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes and mevalonate kinase deficiency, were the first group of systemic autoinflammatory diseases for which a genetic basis was established, between 1999 and 2001. Currently according to the latest report of the international union of immunological societies, 37 separate monogenic disorders were classified as autoinflammatory. In addition to the abovementioned monogenic conditions, we describe Schnitzler's syndrome, a well-defined, acquired autoinflammatory condition without a clear genetic basis. For the purposes of this review, we discuss several conditions defined by the latest consensus process as systemic autoinflammatory diseases. We focus on those disorders where recent studies have contributed to further phenotypic characterization or had an impact on clinical management.
    Source: Rheumatology. - ISSN 1462-0332 (Vol. 58, suppl. 6, Nov. 2019, str. vi31-vi43)
    Type of material - e-article ; adult, serious
    Publish date - 2019
    Language - english
    COBISS.SI-ID - 58561539

    Link(s):

    https://academic.oup.com/rheumatology/article-pdf/58/Supplement_6/vi31/31139742/kez448.pdf
    DiRROS - Digital repository of Slovenian research organizations
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