Adulter Morbus Still, Schnitzler-Syndrom und autoinflammatorische Syndrome im Erwachsenenalter

Lamprecht, Peter

doi:10.1007/s00393-009-0490-y

Cited by 5 publications

(5 citation statements)

References 26 publications

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“…Adult-onset Still's disease (AOSD) is a rare acquired autoinfl ammatory syndrome [ 1 ] . In a retrospective study from Western France, the incidence was reported to be 0.16/100,000 [ 2 ] .…”

Section: Discussionmentioning

confidence: 99%

“…The pathogenesis of AOSD has not been fully elucidated; a hereditary predisposition has been suggested. The disease is characterized by a cytokine imbalance that involves members of the interleukin (IL)-1 family, with elevated IL-18 and IL-1 beta serum levels during times of disease activity [ 1 ] .…”

Section: Discussionmentioning

confidence: 99%

“…AOSD is a diagnosis of exclusion that requires a comprehensive diagnostic workup. The diagnostic criteria most commonly used are those proposed by Yamaguchi et al [ 3 ] ( Table 1 ) [ 1 ] .…”

Section: Discussionmentioning

confidence: 99%

“…Infections, malignancies, other autoimmune diseases as well as hereditary autoinfl ammatory syndromes and Schnitzler's syndrome must be considered in the differential diagnosis. Apart from Still's disease, the latter is among the most common acquired autoinfl ammatory syndromes in adults [ 1 ] . Schnitzler's syndrome is characterized by chronic urticaria, monoclonal gammopathy and at least two other minor criteria such as intermittent fever, arthralgia, bone pain, lymphadenopathy, hepatosplenomegaly, elevated bone density, elevated ESR, and/or leukocytosis [ 8 ] .…”

Section: Discussionmentioning

confidence: 99%

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Rash, fever and arthralgia in a 19‐year‐old woman

Höllweger

Schmuth

Nguyen

2018

J Deutsche Derma Gesell

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Eine 19-jährige Patientin stellt sich aufgrund eines seit zirka einer Woche bestehenden, wenig juckenden, flüchtigen Exanthems an den Oberschenkeln, Knien, Armen und Handrücken vor. Seit drei Tagen klagt sie über rezidivierende Fieberschübe (> 39°C), Myalgien, Arthralgien, vor allem an den Finger-und Zehengelenken, sowie starke Halsschmerzen. Ein rezenter Auslandsaufenthalt sowie Vorerkrankungen werden verneint. Die Patientin hat keine Medikamente vor Auftreten der Beschwerden eingenommen. Die Familienanamnese ist unauffällig.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Rash, fever and arthralgia in a 19‐year‐old woman

Höllweger

Schmuth

Nguyen

2018

J Deutsche Derma Gesell

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“…Weitaus seltener manifestiert sich die Erkrankung erst im Erwachsenenalter, meist um das 30. Lebensjahr[16]. Im Serum lassen sich bei erwachsenen Patienten erhöhte IL-18-und IL-1β-Konzentration nachweisen, die möglicherweise für die typischen rezidivierenden Entzündungsschübe pathogenetisch relevant sind.…”

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Autoinflammatorische Erkrankungen als Ursache von Wundheilungsstörungen

et al. 2011

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Ulcerations of the skin and mucosal membranes are a common feature of autoinflammatory diseases. They can give raise to chronic wound healing defects and should be considered in the differential diagnosis of chronic skin ulcers. The increased activation of the innate immune system in the absence of an apparent provocation for inflammation is a hallmark of autoinflammatory diseases. Mutations and alterations of signaling pathways regulating the innate immune response to physical trauma/tissue damage result into an unrestrained activation of the inflammasome, which leads to increased activation of Interleukin-1. Uncontrolled recruitment and activation of myeloid effector cells within the wound site lead to the release of potent proteases that cause the degradation of structural components of the skin. The majority of these diseases respond well to immunosuppressive and immunomodulatory treatment regimes. Therapeutic resistance converts the acute inflammatory response into a chronic and non-resolving inflammatory process that leads to tissue degeneration. In this article we will focus on the review of those autoinflammatory diseases that often display ulcerative cutaneous and aphthous lesions including pyoderma gangrenosum, Behçet disease, PAPA syndrome and hyperimmunoglobulinemia D with periodic fever syndrome (HIDS). Furthermore, the article will be complemented by an overview of those inflammatory diseases that are associated with non-ulcerative cutaneous manifestations.

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