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Still-Syndrom des Erwachsenen

Adult onset still’s disease — a review of eight cases with discussion of the diagnostic value of serum ferritin levels

Ein Bericht über acht Fälle unter besonderer Berücksichtigung der diagnostischen Wertigkeit des Ferritins

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Zusammenfassung

□ Hintergrund

Das Still-Syndrom des Erwachsenen ist eine seltene entzündlich-rheumatische Erkrankung mit systemischer Beteiligung unbekannter Ätiologie. Leitsymptome sind Fieber, Arthralgien/ Arthritiden und ein stammbetontes, meist makulöses Exanthem.

□ Eigenes Krankengutund Ergebnisse

Von 1980 bis 1996 sind an der eigenen Einrichtung acht Patienten (drei Männer, funf Frauen) mit adultem Still-Syndrom diagnostiziert und behandelt worden. Der Zeitraum zwischen erstem Symptom und Diagnose variierte von zwei bis 86 Monaten. Dies ist auf das Fehlen spezifischer laborchemischer Marker und die vielen differential diagnostischen Möglichkeiten, die ausgeschlossen werden müssen, zurückzuführen. Der in den letzten Jahren in der Literatur beschriebene erhöhte Serumferritinspiegel bei dieser Erkrankung fand sich bei sechs unserer acht Patienten und ist im akuten Stadium ein zusätzlicher Anhaltspunkt für die Diagnose. Unter effektiver Therapie normalisierte er sich rasch. Neben dem Stellen wert des Serumferritins als Diagnose- und Aktivitätsparameter werden unsere therapeutischen Strategien diskutiert und mit den Literaturangaben verglichen.

□ Schlußfolgerung

Bei unklarem Fieber in Verbindung mit Exanthem und Arthralgien sollte ein adultes Still-Syn drom in die Differential diagnose einbezogen werden, dem Patienten können dadurch invasive diagnostische Eingriffe erspart werden. Im akuten Stadium ist ein erhöhter Serumferritinspiegel ein zusätzlicher Hinweis auf das Vorliegen eines Still-Syndroms.

Summary

□ Background

Adult onset Still’s disease (AOSD) is an uncommon, systemic, inflammatory disorder of unknown etiology, characterized by the trial offever, arthritis and rash.

□ Patients and Results

We describe 8 cases of AOSD (3 male, 5 female) diagnosed and treated in the Department of Rheumatology from 1980 to 1996. The delay in reaching a firm diagnosis was between 2 and 86 months, due to both lack of specific serum markers and the abundance of possible differential diagnoses. Our therapeutic strategies and results are presented and the value of obtaining serum ferritin levels for both diagnosis and follow-up studies is discussed. The patients data are compared to those of the world’s literature on AOSD.

□ Conclusion

The differential diagnosis of fever of unknown origin should always include AOSD, because these patients could be spared from invasive and unnecessary diagnostic measures. Increased serum ferritin levels are of particular value in the diagnosis of acute AOSD and the normalization of the serum ferritin value is a reliable indicator of therapeutic success.

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Authors and Affiliations

  1. Klinik für Innere Medizin IV - Nephrologie, Rheumatologie, Osterologie, Pneumologie der Friedrich-Schiller-Universität Jena, Jena, Deutschleend

    Bettina Mock, Gert Hein & Günter Stein

  2. Institut für Klinische Immunologie der Universität, Erlanger Allee 101, D-07747, Jena

    Bettina Mock

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  1. Bettina Mock
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  2. Gert Hein
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  3. Günter Stein
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Mock, B., Hein, G. & Stein, G. Still-Syndrom des Erwachsenen. Med. Klin. 92, 515–520 (1997). https://doi.org/10.1007/BF03044924

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