Adult-onset Still’s disease – Ist ein hoher Ferritinspiegel richtungweisend für die Differenzialdiagnose? 4 Fallbeschreibungen und Literaturübersicht

Zusammenfassung

Es werden 4 Patienten mit dem seltenen Krankheitsbild eines Morbus Still des Erwachsenen vorgestellt. Die Diagnosekriterien nach Yamaguchi waren erfüllt: Fieber, Arthralgien, Exanthem, Pharyngitis, Transaminasenerhöhung und Leukozytose. Der klinische Verlauf und die Schwierigkeiten der Differenzialdiagnose werden dargestellt. Bei erhöhtem Ferritinspiegel sind zunächst Erkrankungen, die mit einer Eisenüberladung einhergehen, auszuschließen. Liegt keine Eisenverwertungsstörung vor, sind vor allem Lymphome, HIV und Hämophogozytose-Syndrome sowie Drug-Induced Hypersensitivity Syndrom (DIHS, DRESS) in die Differenzialdiagnose einzubeziehen. An Hand der Literatur wird versucht, das extrem erhöhte Serumferritin als Makrophagenprodukt darzustellen. Die Höhe des Ferritinspiegels scheint einerseits von der Aktivität der Erkrankung abzuhängen, andererseits ist der Zeitpunkt der Bestimmung innerhalb des Krankheitsverlaufes entscheidend.

Abstract

A total of 4 patients with the rare adult-onset Still’s disease are presented. The diagnostic criteria of Yamaguchi were fulfilled in all patients: fever, arthralgia, rash, sore thoat, increase of transaminases and leukocytes. The clinical history and the difficulties of the differential diagnosis are described. In cases with high ferritin levels, other conditions causing hyperferritinaemia as well as lymphoma, HIV, hemophagocytosis and the rare drug-induced hypersensitivity syndrome (DIHS, DRESS) should be excluded. On the basis of literature reports, a discussion is presented about hyperferritinaemia as a product of macrophages. On the one hand, the level of ferritin seems to be dependent on the disease activity while, on the other hand, in particular it also depends on the time of examination.

Schlüsselwörter

Adult onset Still’s disease - Hyperferritinämie - Makrophagenaktivierung - glykolysiertes Ferritin - nicht klassischer lysosomaler Sekretionsweg

Key words

adult onset Still’s disease - hyperferritinaemia - macrophage activation - glycosylated human ferritin - non-classical lysosomal secretory pathway

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