Hamaguchi Yasuhito, Kuwana Masataka, Hoshino Kana, Hasegawa Minoru, Kaji Kenzo, Matsushita Takashi, Komura Kazuhiro, Nakamura Motonobu, Kodera Masanari, Suga Norihiro, Higashi Akira, Ogusu Koji, Tsutsui Kiyohiro, Furusaki Akira, Tanabe Hiroshi, Sasaoka Shunsuke, Muro Yoshinao, Yoshikawa Mika, Ishiguro Naoko, Ayano Masahiro, Muroi Eiji, Fujikawa Keita, Umeda Yukihiro, Kawase Masaaki, Mabuchi Eriko, Asano Yoshihide, Sodemoto Kinuyo, Seishima Mariko, Yamada Hidehiro, Sato Shinichi, Takehara Kazuhiko, Fujimoto Manabu
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
Arch Dermatol. 2011 Apr;147(4):391-8. doi: 10.1001/archdermatol.2011.52.
To clarify the association of clinical and prognostic features with dermatomyositis (DM)-specific autoantibodies (Abs) in adult Japanese patients with DM.
Retrospective study.
Kanazawa University Graduate School of Medical Science Department of Dermatology and collaborating medical centers. Patients A total of 376 consecutive adult Japanese patients with DM who visited our hospital or collaborating medical centers between 2003 and 2008.
Clinical and laboratory characteristics of adult Japanese patients with DM and DM-specific Abs that include Abs against Mi-2, 155/140, and CADM-140.
In patients with DM, anti-Mi-2, anti-155/140, and anti-CADM-140 were detected in 9 (2%), 25 (7%), and 43 (11%), respectively. These DM-specific Abs were mutually exclusive and were detected in none of 34 patients with polymyositis, 326 with systemic sclerosis, and 97 with systemic lupus erythematosus. Anti-Mi-2 was associated with classical DM without interstitial lung disease or malignancy, whereas anti-155/140 was associated with malignancy. Patients with anti-CADM-140 frequently had clinically amyopathic DM and rapidly progressive interstitial lung disease. Cumulative survival rates were more favorable in patients with anti-Mi-2 compared with those with anti-155/140 or anti-CADM-140 (P < .01 for both comparisons). Nearly all deaths occurred within 1 year after diagnosis in patients with anti-CADM-140. Conclusion Dermatomyositis-specific Abs define clinically distinct subsets and are useful for predicting clinical outcomes in patients with DM.
明确日本成年皮肌炎（DM）患者中临床及预后特征与DM特异性自身抗体（Abs）之间的关联。
回顾性研究。
金泽大学医学研究生院皮肤科及合作医疗中心。患者：2003年至2008年间连续就诊于我院或合作医疗中心的376例日本成年DM患者。
日本成年DM患者的临床和实验室特征以及DM特异性Abs，包括抗Mi-2、抗155/140和抗CADM-140。
在DM患者中，抗Mi-2、抗155/140和抗CADM-140的检出率分别为9例（2%）、25例（7%）和43例（11%）。这些DM特异性Abs相互排斥，在34例多发性肌炎患者、326例系统性硬化症患者和97例系统性红斑狼疮患者中均未检出。抗Mi-2与无间质性肺病或恶性肿瘤的典型DM相关，而抗155/140与恶性肿瘤相关。抗CADM-140患者常患有临床无肌病性DM和快速进展性间质性肺病。抗Mi-2患者的累积生存率高于抗155/140或抗CADM-140患者（两项比较P均<0.01）。抗CADM-140患者几乎所有死亡均发生在诊断后1年内。结论：DM特异性Abs可界定临床上不同的亚组，有助于预测DM患者的临床结局。
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