High Serum Ferritin in Adult-Onset Still's Disease

Peter Jandus, Wei Wang, Michael Seitz, Felix Wermelinger, Hans-Peter Kohler

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DOI: 10.4236/ijcm.2010.12016                

Abstract

In August 2009, a 28-year-old Spanish woman was referred in reduced general condition after at least four medical consultations with a 2 week history of remittent fever, night sweats, exanthema and intermittent arthralgias. Noticeable in the clinical examination was only an urticarial rash on the trunk and extremities and body temperature of 39.6°C. Laboratory tests: WBC with a slight left shift (32%), mild thrombocytopenia (128 G/l), CRP 78 mg/l (N: < 5), elevated transaminases [ASAT 67 U/l (N: 10-37), ALAT 45 U/l (N: 30-65)]. On the fifth day maximum values for ASAT 322 U/l and ALAT 378 U/l were reached. During hospitalisation recurrent fever up to 39.0°C has been documented associated with arthralgias and myalgias. An infection with blood cultures and serology tests was excluded. Rheumatology analysis, urine analysis, chest X-ray, and abdominal ultrasound were inconspicuous. A serum ferritin level of 5493 μg/l (N: 23-110) was detected with peak four days later (8530 μg/l). AOSD was suspected and prednisolone (1 mg/kg body weight) started. The patients recovered rapidly. Transaminases (ASAT 47 U/l, ALAT 149 U/l), CRP (34 mg/l), and ferritin level (1969 μg/l) regressed within 3 days. 3 months later, prednisolone was discontinued; she presented fever (40°C), myalgias, macular rash on the trunk, sore throat and oligoarthritis. Laboratory tests including WBC were without pathologic findings except for transaminases (ASAT 83 U/l, ALAT 53 U/l), CRP (370 mg/l) and ferritin (7434 μg/l). An infectious process was excluded. After resumption of prednisolone 10 mg daily, quick relief of symptoms occurred. Given the short time of relapse, immunosuppressive therapy with cyclosporin was initiated (4 mg/kg body weight). Since that time the patient remained symptom free for over 8 months.

Keywords

High Ferritin, AOSD, Fever, Arthralgia, Rash

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	M. Yamaguchi, A. Ohta, T. Tsunematsu, R. Kasukawa, Y. Mizushima, H. Kashiwagi,S. Kashiwazaki, K. Tanimoto ,Y. Matsumoto, T. Ota, et al., “Preliminary Criteria for Clas-sification of Adult Still’s Disease,” Journal of Rheuma-tology, Vol. 19, No. 3, Mary 1992, pp. 424-430.
[2]	B. Fautrel, “Adult-Onset Still Disease,” Best Practice & Research Clinical Rheumatology, Vol. 22, No. 5, 2008, pp. 773-792.
[3]	V. Bagnari, M. Colina, G. Ciancio, M. Govoni and F. Trotta, “Adult-Onset Still’s Disease,” Rheumatol Interna-tional, Vol. 30, No. 7, 2009, pp. 855
[4]	B. Fautrel, “Ferritin Levels in Adult Still’s Disease: Any Sugar?” Joint Bone Spine, Vol. 69, No. 4, 2002, pp. 355-357.
[5]	B. Fautrel, G. Le Mo?l, B. Saint-Marcoux, P. Taupin, S. Vignes, S. Rozenberg, et al., “Diagnostic Value of Fer-ritin and Glycosylated Ferritin in Adult Onset Still’s Dis-ease,” Journal of Rheumatol, Vol. 28, No.2, 2001, pp. 322-329.
[6]	C. Van Reeth, G. Le Mo?l, Y. Lasne, M. C. Revenant, J. Agneray, M. F. Kahn, et al., “Serum Ferritin and Isoferritins are Tools for Diagnosis of Active Adult Still’s Disease,” Journal of Rheumatology, Vol. 21, No.5, 1994, pp. 890-895.
[7]	T. Zeng, Y. Q. Zou, M. F. Wu and C. D. Yang, “Clinical Features and Prognosis of Adult-Onset Still’s Disease: 61 Cases from China,” Journal of Rheumatology, Vol. 36, No. 5, 2009, pp. 1026-1031.
[8]	S. Vignes, G. Le. Mo?l, B. Fautrel, B. Wechsler, P. Godeau and J-C. Piette, “Percentage of Glycosylated Serum Ferritin Remains Low throughout the Course of Adult Onset Still’s Disease,” Annals of the Rheumatic Diseases, Vol. 59, No.5, 2000, pp. 347-350.
[9]	L. Fardet, P. Coppo, A. Kettaneh, M. Dehoux, J. Cabane and O. Lambotte, “Low Glycosylated Ferritin, a Good Marker for the Diagnosis of Hemophagocytic Syn-drome,” Arthritis & Rheumatism, Vol. 58, No.5, 2008, pp. 1521-1527.
[10]	C. Masson, X. Le Loet, F. Liote, J. J. Dubost, M. C. Boissier, L. Perroux-Goumy, et al., “Comparative Study of 6 Types of Criteria in Adult Still’s Disease,” Journal of Rheumatology, Vol. 23, No. 3, 1996, pp. 495-497.
[11]	J. J. Cush, T. A. Jr. Medsger, W. C. Christy, D. C. Herbert and L. A. Cooperstein, “Adult Onset Still’s Disease. Clinical Course and Outcome,” Arthritis & Rheumatism, Vol. 30, No. 2, 1987, pp. 186-194.
[12]	J. J. Calabro and A. V. Jr. Londino, “Adult Onset Still’s Disease,” Journal of Rheumatology, Vol. 13, 1986, pp. 827-828.
[13]	B. Fautrel, E. Zing, J. L. Golmard, G. Le Moel, A. Bissery, C. Rioux, et al., “Proposal for a New Set of Classification Criteria for Adult-Onset Still Disease,” Medicine, Vol. 81, No. 3, 2002, pp. 194-200.