Vol.22 No.3

Case Report

Leukocytapheresis (LCAP) for treating refractory adult-onset Still’s disease (AOSD)

Authors

Yasumori Izumi1 , Takahiro Mori1 , Makiko Matsuo1 , Yasushi Koga1 , Tadayoshi Ohno1 , Taiichiro Miyashita1 , Osamu Sasaki2 , Hironori Ezaki1 , Kiyoshi Migita1

  • Department of General Internal Medicine and Rheumatology, NHO National Nagasaki Medical Center, 2-1001-1 Kubara, Omura, 856-8652, Japan
  • Department of Nephrology, NHO National Nagasaki Medical Center, 2-1001-1 Kubara, Omura, 856-8652, Japan
Received:

26 April 2011

Accepted:

26 September 2011

Published online:

22 October 2011

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Abstract

This is the first report on the efficacy of leukocytapheresis (LCAP) in a patient with refractory systemic-type adult-onset Still’s disease (AOSD). A 17-yearold Japanese woman with AOSD who had been treated with prednisolone and cyclosporine A presented with relapse of typical systemic AOSD, including high fever, rash, and liver dysfunction. Steroid pulse therapy (methylprednisolone 500 mg/day) was performed, which failed to stabilize the disease. Therefore, LCAP (twice/week for a total of five courses) was introduced in combination with high-dose steroids plus cyclosporin A. Elevated levels of serum ferritin and transaminases and neutrophil CD64 expression normalized after the patient’s disease was successfully controlled by the induction of LCAP. In this case, elevated levels of interleukin (IL)-1β and IL-18 were normalized after LCAP induction, suggesting that LCAP treatment modulates the deregulated cytokine-mediated inflammatory responses observed in AOSD. Our clinical observations suggest that LCAP may be beneficial for flareup of systemic manifestations of AOSD refractory to conventional treatment, including high-dose steroids and immunosuppressants.

Key words

Adult-onset Still’s disease - CD64 - Interleukin-1 - Leukocytapheresis