Original Article

Efficacy of high-dose intravenous immunoglobulin therapy in Japanese patients with steroid-resistant polymyositis and dermatomyositis

Authors

Eizo Saito¹, Takao Koike², Hiroshi Hashimoto³, Nobuyuki Miyasaka⁴, Yasuo Ikeda⁵, Masako Hara⁶, Hidehiro Yamada⁷, Tadashi Yoshida⁸, Masayoshi Harigai⁶, Yoichi Ichikawa⁹ and Additional members of the GB-0998 Study Group

1. Department of Rheumatology, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku Tokyo, 153-8515, Japan
2. Department of Medicine II, Hokkaido University Graduate School of Medicine, Kita 15 Nishi 7, Kita-ku, Sapporo Hokkaido, 060-8638, Japan
3. Division of Rheumatology, Department of Internal Medicine, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku Tokyo, 113-0033, Japan
4. Department of Medicine & Rheumatology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku Tokyo, 113-8519, Japan
5. Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku Tokyo, 160-8582, Japan
6. Institute of Rheumatology, Tokyo Women’s Medical University, 10-22 Kawadacho, Shinjuku-ku Tokyo, 162-0054, Japan
7. Division of Rheumatology and Allergy, Department of Internal Medicine, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki Kanagawa, 216-0015, Japan
8. Department of Pathophysiology, Faculty of Pharmaceutical Science, Hoshi University, 2-4-41 Ebara, Shinagawa-ku Tokyo, 142-8501, Japan
9. St. Joseph’s Hospital, 28 Midorigaoka, Yokosuka Kanagawa, 238-8502, Japan

Received:

4 June 2007

Accepted:

26 September 2007

Published online:

25 January 2008

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Abstract

Intravenous immunoglobulin (IVIG) therapy was administered to 15 patients who were refractory to traditional steroid therapy [eight with polymyosis (PM), seven with dermamyosis (DM)] to evaluate its efficacy. Serum creatine kinase (CK) significantly decreased from week 1, and manual muscle test scores (MMT) and activities of daily living (ADL) significantly increased from week 2. Efficacy rates were 93.3% (14/15 patients) as assessed using the MMT score, 80.0% (12/15 patients) using the ADL score, and 100% (15/15 patients) using the serum CK level. When changes in the serum CK level over two four-week periods, one before IVIG therapy (from week -4 to week 0) and one after IVIG therapy (from week 0 to week 4), were transformed to natural logarithms, the four-week change after IVIG therapy was significantly greater than that before IVIG therapy. The estimated duration of the serum CK level remaining normal in 50% of the patients after IVIG therapy was 334.5 days. Adverse reactions were observed in seven of 16 patients (43.8%) during the study period, but none of the adverse reactions were considered to be serious or required emergency treatment. In conclusion, the present study indicates that IVIG therapy is effective for steroid-resistant PM/DM.

Key words

Dermatomyositis - Intravenous immunoglobulin - Polymyositis steroid resistance - Serum creatine kinase level