Abstract
Adult-onset Still’s disease is a rare systemic inflammatory disease of unknown aetiology characterised by typical symptoms including daily high spiking fever, evanescent salmon-pink rash, sore throat, arthritis/arthralgias and polyserositis. The laboratory findings usually show neutrophilic leucocytosis, seronegativity and raised serum transaminases. We describe six typical cases. All of them had serum ferritin above 5,000 µg/L. Although there are few theories about the origin of the high ferritin level, an extremely high serum ferritin above 5,000 µg/L should be the main diagnostic tool of adult-onset Still’s disease.
This is a preview of subscription content, log in via an institution to check access.
References
Yamaguchi M, Ohta A, Tsunematsu T et al (1992) Preliminary criteria for classification of adult Still’s disease. J Rheumatol 19:424–430
Mirahmadi KS, Paul WL, Winer RL et al (1977) Serum ferritin levels. JAMA 238:601–603
Worwood M (1986) Serum ferritin. Clin Sci 70:215–220
Prieto J, Barry M, Sherlock S (1965) Serum ferritin in patients with iron overload and with acute and chronic liver diseases. Gastroenterology 68:526–533
Schwarz-Eywill M, Heilig B, Bauer H, Breitbart A, Pezzutto A (1992) Evaluation of serum ferritin as a marker for adult Still’s disease activity. Ann Rheum Dis 51:683–685
Hoshino T, Ohta A, Yang D, Kawamoto M, Kikuchi M, Inoue Y et al (1998) Elevated serum interleukin 6, inteferon-y, and tumor necrosis factor α levels in patients with adult Still’s disease. J Rheumatol 25:396–398
Rogers JT, Bridges KR, Durmowicz GP, Glass J, Auron PE, Munro HN (1990) Translational control during the acute phase response. Ferritin synthesis in response to interleukin-1. J Biol Chem 265:14572–14578
Kawashima M, Yamamura M, Taniai M et al (2001) Levels of interleukin-18 and its binding inhibitors in the blood circulation of patients with adult-onset Still’s disease. Arthritis Rheum 44:550–560
Van Reeth C, le Moel G, Lasne Y, Revenant MC, Agneray J, Kahn MF et al (1994) Serum ferritin and isoferritins are tools for diagnosis of active adult Still’s disease. J Rheumatol 21:890–895
Kondo H, Saito K, Grasso JP, Aisen P (1988) Iron metabolism in the erythrophagocytosing Kupffer cell. Hepatology 8:32–38
Sibille JC, Kondo H, Aisen P (1988) Interactions between isolated hepatocytes and Kupffer cells in iron metabolism: a possible role for ferritin as an iron carrier protein. Hepatology 8:296–301
Campbell CH, Solgonick RM, Linder MC (1989) Translational regulation of ferritin synthesis in rat spleen: effects of iron inflammation. Biochem Biophys Res Commun 160:453–459
Fautrel B (2002) Ferritin levels in adult Still’s disease: any sugar? Joint Bone Spine 69:355–357
Kirino Y, Takeno M, Iwasaki M et al (2005) Increased serum HO-1 in hemophagocytic syndrome and adult-onset Still’s disease: use in the differential diagnosis of hyperferritinemia. Arthritis Res Ther 7:R616–R624
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Novak, S., Anic, F. & Luke-Vrbanić, T.S. Extremely high serum ferritin levels as a main diagnostic tool of adult-onset Still’s disease. Rheumatol Int 32, 1091–1094 (2012). https://doi.org/10.1007/s00296-011-1834-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-011-1834-x